BSG Conference 2019 - Programme & Abstract book

Annual Conference 2019

PROGRAMME & ABSTRACT BOOK

Main Partner:

Wednesday 27th & Thursday 28th February 2019 Royal College of Physicians, London

Contents Contents

Welcome to the BSG Conference 2019

Welcome to BSG

3

General Info

4-5

BSG Update

5

About the BSG

6-7

Keynote Speakers

8 - 11

Main Conference Programme

12 - 19

NSF Programme

20

Posters

21 - 25

Sponsors

26

Exhibitor floorplan

27

Abstracts - Free Papers

29 - 42

Abstracts - poster

43 - 84

Abstracts - NSF

85 - 88

BSG 2020

89

Note Paper

90 - 94

Welcome to the British Sarcoma Group Annual Conference 2019 in the auspicious surroundings of the Royal College of Physicians.

We are delighted that you have come to join us for what promises to be an excellent conference. Our aim is to update and stimulate you, and give you the opportunity to catch up with friends and colleagues.

We chose key topics for the programme including adjuvant chemotherapy for soft tissue sarcoma, immunotherapy, rare and spinal sarcomas, service development, fibromatosis, GIST and difficult palliative problems.

This is a multidisciplinary conference and we are pleased to host the National Sarcoma Forum, a key event for nurses and allied health professionals and key sessions on extended practice roles and rehabilitation. Thanks to Julie Woodford and Abbie McCarthy for their efforts in bringing this part of the programme together.

The programme has been substantially enriched by contributions from the sarcoma community at home and abroad. Please welcome and talk to our keynote speakers who bring new perspectives. This year brought more abstract submissions than ever before, and we are looking forward to hearing from researchers, old and young.

Thanks to all of you who have contributed to developing and delivering the conference, to our hosts, our sponsors and the team at Echo events, without whom this would not have been possible.

Enjoy!

Craig Gerrand, Beatrice Seddon, Andrew Hayes, Ian Judson, Julie Woodford and Abigail McCarthy Scientific Committee - BSG Conference 2019

Page 2

Page 3

British Sarcoma Update

General Info REGISTRATION The registration desk will be located in the foyer for both days of the conference.

REFRESHMENTS Refreshments and lunch on both days will be served in the exhibition room.

EXHIBITORS & CHARITIES Sponsor exhibitors will be located in the Osler and Long Room along with charity supporters.

CONFERENCE SESSIONS Will take place in the Wolfson Theatre.

Parallel sessions take place in the Dorchester Library.

PRESENTERS Presenters must have their talks loaded to the show laptops well in advance of their lecture. There will be no allowance for individuals to load their slides at the time of the presentation or use their own laptop. On arrival please report to the registration desk where a member of the events team will direct you to the AV team.

POSTER EXHIBITION Posters will be displayed in the Council Chamber. Access to the poster presentations will be available from Wednesday 27th Feb at 9am. Posters must be taken down at the close of conference. Any posters left after the

conference will be discarded.

ABSTRACTS Abstracts for oral talks and poster displays can be found on pages 29 - 88.

THE CONFERENCE DINNER The BSG Conference Dinner will take place at the Royal College of Physicians on Wednesday 27th February. The evening includes a complimentary glass of prosecco on arrival, a cash bar and a three course meal. Dress code is informal.

CPD CREDITS The conference has applied for continuing professional development points with the British Orthopaedic Association.

Attendance certificates will be emailed after the event.

AWARDS CEREMONY The awards ceremony will take place at the end of conference on Thursday 28th Feb. Awards will be presented for: • BSG Special Award • Poster Award

WIFI Wifi is complimentary and there is no password needed. To access please select the RCP Network and enter your email address to log on.

INSURANCE The organisers do not accept liability for individual medical, travel or personal insurance and participants are advised to make their own arrangements with regards to insurance. The organisers reserve the right to alter or cancel, without prior notice, the conference or any arrangements relating directly or indirectly to it. The organisers shall not be liable for any loss damage, expenditure or inconvenience caused as a result of such alterations or cancellations. The content of this programme is correct at the time of publishing. However, the organisers reserve the right to alter or cancel the programme if necessary.

EVALUATION An evaluation form has been included in the delegate pack. We would be grateful if all delegates could complete the form and hand back to a member of the conference team.

Page 4

BSG has had another good year, and we have consolidated the reorganisation of recent years.

This has allowed us to remain compliant with many regulations and, most importantly, viable.

We are delighted to report that we now have 200 registered members!

The Conference is the main event of the year and we are extremely grateful to those who have worked so hard to bring it together. There is a rich and varied programme which reflects the work that you are doing as a community.

As well as Conference, this year BSG has produced guidelines on the ultrasound screening of soft tissue masses, and held a workshop for Sarcoma MDTs, at which many of you shared the issues we face in delivering a good service to increasing numbers of patients. We have made progress with our SACT panel, and responded to the RCOG proposals about morsellisation of uterine fibroids, some of which sadly turn out to be sarcomas.

As ever there are considerable pressures on all of us trying to provide care for sarcoma patients.

Many of us work in imperfect environments, struggling to look after patients with challenging problems and in a system with limits to what it can provide. The British Sarcoma Group is your organisation and there is a great deal more we can do to educate, train, research and improve services for sarcoma patients. Your contributions and ideas are very welcome – BSG can be a platform to support your initiatives. As ever, let any one of the Trustees know if you want to get involved – we need your enthusiasm, time and effort!

Craig Gerrand BSG President

Page 5

About the British Sarcoma Group The British Sarcoma Group is registered as a charitable incorporated organisation (CIO) by the Charity Commission. Our registration number is 1154928. A CIO has the legal privileges of a full charity together with aspects of legal protection and limited liability which apply to companies.

THE AIMS OF THE BRITISH SARCOMA GROUP ARE:

MEMBERSHIP All clinicians, nurses and supporting professionals who treat patients with sarcoma in England, Wales, Scotland and Northern Ireland are eligible to join the British Sarcoma Group – membership is free and gives the following benefits: Membership Benefits •

Become part of an engaged community of fellow specialists seeking to improve sarcoma treatment

•

Access member surveys and research

•

Help influence national guidelines on best practice

•

Join a national network of those working to treat sarcoma

•

Benefit from educational opportunities

•

Receive regular newsletters and updates from us

1. To promote the education of medical, nursing and healthcare professionals in the UK concerned with the treatment and care of patients with bone and soft tissue sarcomas through the provision of information, training, and the identification of best practice 2. To promote excellence in the healthcare and treatment of patients with bone and soft tissue sarcoma, through enabling specialist healthcare professionals to identify and share best practice 3. To promote and support research, including the publication of results to support the development of more effective treatment and care of patients with bone and soft tissue sarcomas 4. To improve the knowledge and understanding of sarcoma amongst the medical profession and general public.

HOW TO JOIN Becoming a member of the British Sarcoma Group is quick and easy – simply fill in the BSG Membership Form and return to support@britishsarcomagroup.org.uk and we will set up your membership.

FUNDING The BSG’s income comes via the annual conference. Sponsorship and delegate registrations fund the cost of running the conference, with any small surplus allocated to support other BSG projects such as production of the BSG guidelines and to secure the conference venue for the following year.

Administration of the British Sarcoma Group Routine administration of the charity and conference management is undertaken by the Secretariat who can be contacted at support@britishsarcomagroup.org.uk or on 0191 244 2581.

TRUSTEES The BSG is managed by a Board of Trustees with support from advisory groups relating to specific projects.

The Trustees are: • President - Mr Craig Gerrand (Consultant Orthopaedic Surgeon, Royal National Orthopaedic Hospital, Stanmore) • Dr Adam Dangoor (Medical Oncologist, University Hospitals Bristol NHS Trust) • Professor Ian Judson (Medical Oncologist, Royal Marsden Hospital, London) • Mr Andrew Hayes (Consultant Surgeon, Royal Marsden Hospital, London) • Mrs Anita Pabla (Macmillan Sarcoma Nurse Specialist, Leicester Royal Infirmary) • Dr Beatrice Seddon (Clinical Oncologist, University College London Hospitals) • Dr Owen Tilsley (Clinical Oncologist, Velindre Hospital, Wales) • Dr. Paul Cool (Consultant Orthopaedic & Oncological Surgeon, The Robert Jones and Agnes Hunt Orthopaedic Hospital) • Dr. Martin H Robinson (Honorary Reader in Clinical Oncology Academic Unit Oncology, University of Sheffield, Weston Park Hospital) • Dr Ioanna Nixon (Consultant Clinical Oncologist, Head and Neck / Sarcoma, The Beatson West of Scotland Cancer Center, Glasgow, UK) There are opportunities for BSG members to get involved in advisory and working groups that contribute to particular areas of the BSG’s work. If you are interested, please talk to one of the Trustees or email support@britishsarcomagroup.org.uk

Already a member? You can update your details using the BSG membership form available online at www.britishsarcomagroup.org.uk

Page 6

Page 7

Keynote Speakers Dr Seth Pollack Dr. Seth Pollack is an attending oncologist specializing in sarcoma at the Seattle Cancer Care Alliance, the largest tertiary referral center in the Northwestern United States. He has dual faculty appointments at the University of Washington and Fred Hutchinson Cancer Research Center. The Pollack Lab develops novel immunotherapies for patients with advanced sarcoma, with a particular focus on those sarcomas that express high levels of the cancer testis antigen NY-ESO-1, Synovial Sarcoma and Myxoid/ Round Cell Liposarcoma. Dr. Pollack has developed methods for identifying and expanding NY-ESO-1 specific T cells from the blood of these patients and has led intensive immune monitoring efforts for trials targeting this antigen. Based on data from a phase 0 trial, he designed a newly active multicenter trial of interferon gamma and pembrolizumab for these patients sponsored by the Cancer Immunotherapy Trials Network.

Dr. Pollack has won multiple awards for his research including the SARC Career Development Award, The V Foundation Translational Research Award and the Alliance for Cancer Gene Therapy Award. He was featured in the widely followed audio/cd/ podcast series Research-to-Practice and wrote an invited review for the Journal of Clinical Oncology on immunotherapy and novel therapies for advanced sarcoma. Dr. Pollack received the Sig Kohl Legacy award from the Northwest Sarcoma Foundation for his “outstanding and enduring contribution to the care of sarcoma patients in the Northwest.”

Dr Sebastian Bauer Dr. Bauer received his Medical Degree at the University of Würzburg and his training as Medical Oncologist at the West German Cancer Center in Essen, Germany. He is now directing the Sarcoma Program at the center, which is among the largest in the country with app. 400 new patients per year. His research interests include the elucidation of novel resistance mechanisms in GIST as well as the development of novel sarcoma models.

Prof Bernd Kasper Prof. Bernd Kasper studied Medicine at the University of Heidelberg and received his MD degree from Heidelberg University in 2000. In 2001, he finalised his thesis at the German Cancer Research Centre (DKFZ) in Heidelberg, dealing with new treatment strategies for chronic myelogenous leukaemia patients using the tyrosine kinase inhibitor imatinib. To deepen his training, he stayed in London (Imperial College School of Medicine, Hammersmith Hospital, Department of Haematology), Brussels (Jules Bordet Institute, Medical Oncology Clinic) and South Kerala, India (scholarship of the Deutscher Akademischer Austauschdienst DAAD).

In 2007/2008, he specialized in Internal Medicine and Medical Haematology/ Oncology at the Department of Internal Medicine V at the University of Heidelberg.

Currently, he works together with Prof. Peter Hohenberger at the Sarcoma Unit of the Interdisciplinary Tumor Center Mannheim (ITM) at the Mannheim University Medical Center. He has been chair of the ITM since 2011. Prof. Kasper has a life-time professional dedication to patient care and research in soft tissue sarcomas (STS), desmoid-type fibromatosis (DF) and gastrointestinal stromal tumors (GIST). His clinical practice focuses exclusively on these disease entities. He is head of the study center of the German Interdisciplinary Sarcoma Group (GISG) and is active in national and international study groups (AIO, EORTC). He is the Principal Investigator of several national and international trials on STS, DF and GIST. Currently, he serves as the Secretary of the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) and is member of the German Cancer Society (DKG), DGHO, GISG, European Society for Medical Oncology (ESMO) and Connective Tissue Oncology Society (CTOS).

Prof. Kasper has authored around 100 scientific publications. He is dedicated as board member to patient organizations dealing with STS, DF and GIST on the national and international level such as SPAEN or DTRF.

He is also rooting for Jürgen Klopp to be the first German Premier League Coach to win the title.

Page 8

Page 9

Keynote Speakers Prof David Gourevitch Professor David Gourevitch was appointed as a consultant surgeon in 1992 after completing his surgical training with dual accreditation in thoracic and upper GI/general surgery. Previously, he had worked in Africa (Mzuzu, Malawi, Durban, South Africa and Nqutu, Kwazulu) and written his MD thesis in vascular surgery.

Prof Anna Campbell Professor Anna Campbell is Professor in Clinical Exercise Science at Edinburgh Napier University and has 18 years experience in the field of exercise oncology and has been instrumental in the implementation of evidence based exercise programmes for people affected by cancer across UK.

Originally appointed with a particular interest in upper GI resectional surgery to Sandwell Hospital, his clinical practice was large and encompassed those of the neighbouring hospitals. In addition, he ran a large paediatric surgical service.

She is principal investigator / co-investigator on Macmillan, NIHR, CSO and Breast Cancer Now funded research grants and has published over 40 peer reviewed research papers and three educational book chapters on the subject of cancer and exercise.

His practice was transferred to University Hospitals Birmingham NHS Foundation Trust (UHB) in 2003 when he was asked to lead the upper GI service at the teaching hospital. He subsequently established the Midland Abdominal and Retroperitoneal/Pelvic Sarcoma Unit (MARSU) in 2007 and, together with the Bone Sarcoma Service based at Royal Orthopaedic Hospital (ROH), formed the Birmingham Sarcoma Service.

As key advisor to Macmillan Cancer Care, she worked on the development of the MoveMore programmes in Scotland and the evaluation of the UK wide MoveMore programmes. Professor Campbell is also Director of CanRehab (www.canrehab.co.uk), established in 2007, which is an international provider of training on cancer exercise based rehabilitation for health professionals and fitness specialists.

MARSU continues to expand and operates a multi-specialty unit with other surgical specialties based at UHB. The unit supports local and national sarcoma trials and contributes to the 100,000 Genome Project. It has also established a sarcoma fellowship and has close links to the sarcoma centres in Paris and Milan with whom the unit exchanges training surgeons.

Professor Campbell is part of an international team cancer exercise experts working on updating the evidence based exercise prescription guidelines and the guidelines for the provision of clinical and community based exercise programmes for cancer patients and survivors. The findings will be published in 2019.

Professor Gourevitch has held administrative appointments at UHB and national surgical societies, national committees and the Royal College of Surgeons. He is a non-executive director at the ROH. He continues as the older operating sarcoma surgeon at MARSU.

This talk will review the current hot topics of research in exercise oncology and provide information on the plan and status of the new guidelines on implementation of exercise programmes for cancer patients and survivors.

Page 10

Page 11

AUDITORIUM Welcome to BSG 2019 Craig Gerrand, British Sarcoma Group

ROOM: 08:45 - 08:55

Free Papers 1 - Surgery CHAIRS: Myles Smith

AUDITORIUM

ROOM:

Morning Refreshments (Poster viewing in Council Chamber)

10:45 - 11:15

AUDITORIUM Improving Sarcoma MDTs

ROOM: 12:30 - 13:00

13:00 - 14:00

Lunch (Poster viewing in Council Chambers)

This session was originally scheduled as a keynote lecture from Professor Martin Gore who very sadly passed away

unexpectedly. Professor Gore was a much loved and respected colleague who will be greatly missed. Our thoughts are with his

family and friends at this difficult time.

12:30 - 12:40 Why restructure Sarcoma MDTs? - Craig Gerrand 12:40 - 12:50 Pathways and Protocols - Andrew Hayes 12:50 - 13:00 National Data Collection - Sandra Strauss

Plenary talk: Overview of immunotherapy in sarcoma management today – Seth Pollack Genetic analyses for predicting suitability for immunotherapy in sarcoma – Nishalan Pillay Surgical immunotherapy – ILP and oncolytic viruses - Andrew Hayes Combining immunotherapy and radiotherapy – Shane Zaidi What is available to patients in the UK? – Sandra Strauss Wrap up

11:15 - 11:45 11:45 - 11:55 11:55 - 12:05 12:05 - 12:15 12:15 - 12:25 12:25 - 12:30

ROOM: AUDITORIUM 11:15 - 12:30 Immuno-therapy in Sarcoma CHAIR: Bass Hassan

Introduction - Robin Jones Summary of the evidence – Robin Young How would we deliver this in the UK – Adam Dangoor Panel discussion and questions Wrap up – What should the BSG position be on this? Robin Jones

09:45 - 09:50 09:50 - 10:05 10:05 - 10:20 10:20 - 10:35 10:35 - 10:45

09:45 - 10:45 Adjuvant chemotherapy for soft tissue sarcoma – should we change practice?

CHAIR: Robin Jones

West of Scotland Breast Sarcoma Audit - Naomi Foster

09:35 - 09:45

08:55 - 09:05 Outcomes after hindquarter amputation - Christian Gray Stephens 09:05 - 09:15 Reconstructing lower limbs of the growing child with the Juvenile Tumour System - Christian Gray Stephens 09:15 - 09:25 Inferior vena cava (IVC) leiomyosarcomas involving bilateral renal veins – is surgical resection without reconstruction safe? Dirk Strauss 09:25 - 09:35 Surgical and oncological outcomes of localized radiation-induced sarcoma of bone - Martin Lowe

08:55 - 09:45

ROOM: AUDITORIUM

Registration

08:00 - 08:45

Programme - Day One: Wednesday 27th February 2019

Afternoon Refreshments (Poster viewing in Council Chamber)

16:00 - 16:30

Conference Close

Drinks Reception BSG Conference Dinner

18:15

19:30 20:00

Speaker: David Gourevitch

ROOM: AUDITORIUM 17:45 - 18:15 BSG Keynote Lecture – Surgery for retroperitoneal sarcoma, a personal view CHAIR: Andrew Hayes

16:30 - 16:45 Surgery for sarcomas of the spine – Hanny Anwar 16:45 - 17:00 Surgery for sacral tumours – Will Aston 17:00 - 17:20 Radiotherapy to the spine and sacrum – Beatrice Seddon 17:20 - 17:35 Rehabilitation and life after treatment of spinal tumours – Manish Desai 17:35 - 17:45 Discussion and questions

ROOM: AUDITORIUM 16:30 - 17:45 Local therapy symposium: Spinal and sacral sarcoma CHAIR: Mel Grainger

Epithelioid sarcoma – Spyridon Gennatas Clear cell sarcoma – Mariam Jaffri PEComa – Vasilios Karavasilis Discussion, questions and wrap up – Robin Jones

15:00 - 15:15 15:15 - 15:30 15:30 - 15:45 15:45 - 16:00

ROOM: AUDITORIUM 15:00 - 16:00 Rare sarcoma symposium: Overview and treatment

approaches CHAIR: Robin Jones

14:10 - 14:20 The Breast Angiosarcoma Surveillance Study (BRASS) - Jenny Banks 14:20 - 14:30 Retrospective evaluation of 294 consecutive 18F-FDG PET-CT scans

in gastro-intestinal stromal tumours (GIST) with respect to driver

mutation subtypes and treatment interventions - Bass Hassan 14:30 - 14:40 Radiological and Histopathology alterations following pre operative Radiotherapy in Soft Tissue Sarcomas of the extremities - Nikolaos Memos 14:40 - 14:50 RNA sequencing of Ewing’s sarcoma patient-derived cancer stem-

like cells identifies novel biomarkers of risk and putative targets for therapy - Elizabeth Roundhill 14:50 - 15:00 A Phase 1 Study Of The Poly-Adp Ribose Polymerase (PARP)

Inhibitor, Niraparib (NIR), in combination with Irinotecan (IRN) in

patients with advanced Ewing Sarcoma: Results of SARC025 Arm 2 - Sandra Strauss

See page 20 for the NSF

Sponsored by:

programme

14:00 - 14:10

Incidence and survival of soft tissue sarcoma in England between 2002 and 2016: an analysis from the National Cancer Registration and Analysis Service (NCRAS) - Andrew O Bacon

ROOM: DORCHESTER LIBRARY 14:00 - 17:30 National Sarcoma Forum

ROOM: AUDITORIUM 14:00 - 15:00 Free Papers 2 - The six best CHAIRS: Adam Dangoor & Andrew Hayes

Programme - Day One (Continued)

AUDITORIUM

ROOM:

Clinical trials update CHAIR: Jeremy Whelan

08:35 - 09:35

08.35 - 08.40 Scene Setting - Abby McCarthy & Hannah

Hughes 08.40 - 08.55 Putting preadmission into the sarcoma pathway

what are the gains? - Jill Cooper 08.55 - 09.10 The introduction of a Physiotherapy Advanced

Practitioner highlighting the benefits

Gillian L Cribb 09.10 - 09.25 Introducing The role of a Research Therapist Sherron Furtado 09.25 - 09.35 Open to the floor for discussion

ROOM: DORCHESTER LIBRARY 08:35 - 09:35 Extended Practice Roles

Physical activity is billed as the new wonder drug for cancer helping people overcome fatigue, anxiety and depression, while protecting the heart, lungs and bones. In some cases, being physically active has been shown to slow disease progression, improve survival and reduce the chance of recurrence. For those diagnosed with sarcoma the effects of treatment often limits their ability to return to physical activity. In this session we review the latest evidence and start to ask questions around the barriers to participation, how can we develop the research and evidence base within this domain to improve knowledge and clinical outcome - Dr Anna Campbell

10:50 - 11:00 Desmoid fibromatosis through the patients’ eyes:

time to change the focus and organisation of care? - Olga Husson 11:00 - 11:10 Real-world experience with doxorubicin and

olaratumab in soft tissue sarcomas across South

East England - Spyridon Gennatas 11:10 - 11:20 Clinical relevance of Immune checkpoints and

HLA expression in chondrosarcoma - Ahmad

Al-sukaini 11:20 - 11:30 Outcome of Patients with Localized High-Grade

Osteosarcoma Treated with Mifamurtide Michael Parry 11:30 - 11:40 High-dose chemotherapy for the treatment of

recurrent and primary-refractory Ewing Sarcoma -

Rachael Windsor 11:40 - 11:50 Tumour necrosis is significantly associated with

reduced overall survival after curative resection of GIST - Robert Tyler

ROOM: DORCHESTER LIBRARY 10:50 - 11:50 Free Papers 3 - Chemotherapy CHAIRS: Beatrice Seddon & Charlotte Benson

Morning Refreshments (Poster viewing in Council Chamber)

Service specification - Jeremy Whelan SACT update - Adam Dangoor and Charlotte Benson Proton Update - James Wylie

ROOM: AUDITORIUM 10:50 - 11:50 Symposium: Best Practice in Rehabilitation CHAIR: Abbey McCarthy

10:20 - 10:50

09:35 - 09:50 09:50 - 10:05 10:05 - 10:20

ROOM: AUDITORIUM 09:35 - 10:20 Sarcoma Service updates CHAIR: Craig Gerrand

08:35 - 08:45 NCRI update - Craig Gerrand 08:45 - 09:25 Opening and upcoming trials:

ICONIC - Sandra Strauss

Afatinib chordoma trial – Sandra Strauss

FaR-RMS study - Julia Chisholm

Euro-Ewing 20xx - Bernadette Brennan

rEECur trial – Martin McCabe

STRASS 2 - Dirk Strauss

Sarcoma Assessment Measure - Rachel Taylor

Levatinib study - Quentin Campbell Hewson 09:25 - 09:35 100K genome study update and next steps Adrienne Flanagan

AUDITORIUM

ROOM:

08:30 - 08:35 Welcome to Day 2 Craig Gerrand, British Sarcoma Group

Registration

08:00 - 08:30

Programme - Day Two: Thursday 28th February 2019

Lunch (Poster viewing in Council Chamber)

DORCHESTER LIBRARY

11:50 - 12:00 Diagnostic accuracy of MRI and histology in assess

ment of deep lipomatous tumours - Hayden Snow 12:00 - 12:10 A daily diagnostic multidisciplinary meeting to

reduce time to definitive diagnosis in the context of

primary bone and soft tissue sarcomas - Laura Hartley 12:10 - 12:20 A Rational Approach to Two Week Wait Referrals - Damien McDermott 12:20 - 12:40 Evaluation of telephone clinic service for 2-week rule

referrals for suspected soft tissue sarcomas in a

tertiary centre - Nikolaos Memos 12:40 - 12:50 Measuring what matters: developing the content of

the Sarcoma Assessment Measure (SAM) - Rachel Taylor 12:50 - 13:00 A qualitative exploration of the experiences of

teenagers and young adults with sarcoma - Ana Martins

11:50- 12:50 Free Papers 4 - Service improvement CHAIRS: Julie Woodford & Dirk Strauss

ROOM:

AUDITORIUM GIST – An update on best practice CHAIR: Charlotte Benson Fifty shades of KIT – all we need know to ‘punish’ GIST? - Sebastian Bauer Robotic surgery for GIST - Myles Smith The UK perspective – Charlotte Benson Questions and wrap up

10 minute Break AUDITORIUM

ROOM: 14:50 - 15:50

14:50 – 15:20 15:20 – 15:30 15:30 – 15:40 15:40 – 15:50

15:50 - 16:00 ROOM:

AUDITORIUM

Closing remarks

Close of conference

17:00 - 17:05

17:05

What does inoperable mean? Rob Pollock What can be achieved with chemotherapy in inoperable tumours? Rachael Windsor Definitive radiotherapy - Franel Le Grange Interventional pain management strategies - Ali Mofeez Challenges of achieving good palliation and end of life care - Jane Neerkin

ROOM:

16:00 – 16:10 16:10 – 16:20 16:20 - 16:30 16:30 – 16:45 16:45 – 17:00

16:00 - 17:00 Symposium: Palliation of inoperable pelvic sarcomas CHAIR: Maria Michelagnoli

Living with fibromatosis – Olga Husson Pathology of fibromatosis – Roberto Tirabosco What can be achieved with radiotherapy? Aisha Miah Plenary lecture – The Management of Desmoid Tumors: A Joint Global Evidence-Based Consensus Guideline Approach for Adult and Pediatric Patients - Bernd Kasper

13:50 - 14:00 14:00 - 14:10 14:10 - 14:20 14:20 - 14:50

ROOM: AUDITORIUM 13:50 - 14:50 Symposium: Fibromatosis – an overview and update

CHAIR: Palma Dileo

12:50 - 13:50

ROOM: AUDITORIUM 12:35 - 12:50 National Sarcoma Awareness Project Chandra Chandrasekar

Sponsored by:

Speakers: Dr Spyridon Gennatas,

Dr Nicola Keay and Dr Nasim Ali

ROOM: AUDITORIUM 11:50 - 12:35 Case Based Discussions in Sarcoma CHAIR: David Peake

Programme - Day Two (Continued)

National Sarcoma Forum: Programme

Posters

ROOM 14:00 - 14:10

1. Audit of post op orthopaedic oncology referrals – are outpatient appointments booked within the requested time frame?

Abby McCarthy, RNOH Stanmore

DORCHESTER LIBRARY Welcome and Introductions Christine Millman, Julie Woodford & Anne Mctiernan

Aoife O’Meara, Royal National Orthopaedic Hospital 10. Dermatofibrosarcoma Protuberans (DFSP) in Children: A combined multidisciplinary approach to rare tumors Aseel Sleiwah, Guy’s and St Thomas’ NHS Foundation Trust

14:10 - 14:40 Sarcoma Treatment: Impact on Fertility and Menopause GUEST SPEAKER: Dr Melanie Davies, Consultant Obstetrician and

Gynaecologist - UCLH

2. The Aesthetic approach to anterior abdominal wall tumours Ahmed Emam, Southmead Hospital Bristol

14:40 - 15:10 Making sense of living with and beyond cancer: the ARC cancer

framework GUEST SPEAKER: Dr Clair Le Boutillier, Macmillan Research Associate

3. Diagnostic accuracy and safety of CT guided biopsy of bone and soft tissue lesions performed under general anaesthetic (GA): a single centre experience on 454 patients Alessandro Vidoni, Royal National Orthopaedic Hospital NHS Trust

15:10 - 16:00

FREE PAPERS

15:10 15:20

15:30

15:40 15:50

Your Voice Counts - Listening to Patients - Julie Woodford “The Clinical Nurse Specialist was really a stable point for me to go to for any sort of information or support” – the role of professionals’ expertise and support in patients’ experience - Ana Martins Review of physiotherapy provision at Bone and Soft Tissue Sarcoma Centres across England - Lynsey Green Physiotherapist Led Bone Tumour Surveillance Clinics - Geraint Davies Questions

16:00 - 16:30

Afternoon Refreshments

16:30 Genomics Medicine GUEST SPEAKER: Professor Adrienne Flanagan, NIHR Senior Investigator,

Sarcoma Genomics England Clinical Interpretation Partnership (GeCIP),

Honorary Consultant Histopathologist 17:00

Interactive workshop with the delegates of the NSF to identify the

clinical utility of SAM - Dr Rachel Taylor & Julie Woodford 17:30

Close

18:15

NSF Committee Meeting (Venue to be announced)

National Sarcoma Forum Sponsor:

4. Value of staging in non-conventional appendicular osteosarcoma.

Alessandro Vidoni, Royal National Orthopaedic Hospital NHS Trust 5. Multi-specialty surgical approach to patients with primary chest wall sarcoma A. Bridgeman, Department of Thoracic Surgery, University Hospitals Bristol NHS Foundation Trust

12. The role of Evicel® (fibrin sealant) in bone and soft tissue tumour surgery Benjamin Davies, Royal National Orthopaedic Hospital 13. Trabectedin: Based on 8 years of experience, is it worth offering this chemotherapy to patients without L-type soft tissue sarcomas?

Beth Lambourne, Freeman Hospital NHS

6. Management of epithelioid sarcoma Knight Andrew, Leeds Teaching Hospital NHS Trust

14. Role of Bisphosphonates as Adjuvants of Surgery in Giant Cell Tumor of Spine Chaitanya Dev Pannu, Royal National Orthopaedic Hospital, London

7. Quality Improvement in Sarcoma Diagnostic Pathway Julie Woodford, London Sarcoma Service

15. Sarcoma: A Head To Toe Approach Maniram Ragbir, Newcastle Upon Tyne NHS Foundation Trust

8. A review of the use of Doxorubicin and Olaratumab for advanced soft-tissue sarcoma in the North East Dr Anna Stansfeld, Northern Centre for Cancer Care, Freeman Hospital, Newcastle

16. Cardiac intimal sarcoma- a case report Christine Gray, University Hospitals Birmingham, NHS Foundation Trust

9. Service Evaluation of incidence and management of proximal femoral replacements (PFR) for metastatic bone disease versus sarcoma Page 20

11. Rates of Osteointegration of Hydroxyapatite-Coated Collars in Cemented Massive Endoprostheses Following Revision Surgery Benjamin Davies, Royal National Orthopaedic Hospital

17. The effect of crizotinib on an ALKrearranged inflammatory myofibroblastic tumour- a case report Christine Gray, University Hospitals Birmingham, NHS Foundation Trust

Page 21

18. Comorbidity in Soft Tissue Sarcoma: A Pilot Study Ciaran Grafton-Clarke, University of Leicester

(PEComa): Presentation, Treatment and Outcome in a Single Institution Han Hsi Wong, Cambridge University Hospitals NHS Foundation Trust

19. Pre-operative biopsy practice and accuracy in a Retroperitoneal Sarcoma (RPS) unit Eleanor Pettitt, University of Nottingham 20. Survival Outcomes after Retroperitoneal Sarcoma (RPS) surgery in a single UK centre Eleanor Pettitt, University of Nottingham 21. Complications following excision of bone and soft tissue sarcoma of the extremities and incidence of postoperative Lymphedema Fabio Nicoli, Newcastle upon Tyne Hospitals NHS Trust,UK 22. Treatments for Low Grade Fibromyxoid Sarcoma: Systematic Review and Experience of a Tertiary Cancer Centre Florence Chamberlain, The Royal Marsden Hospital NHS Foundation Trust 23. Use of Regorafenib in Patients with Gastro-Intestinal Stromal Tumour (GIST): Experience of Toxicity Management in a Tertiary Cancer Centre Constance Williams-Sharkey, The Royal Marsden Hospital

29. Bayley Walker Proximal Humeral Reconstruction – The Stanmore Experience Heledd Havard, Royal National Orthopaedic Hospital 30. Sarcoma UK Support Line - Using patients voices to drive change Helen Stradling, Sarcoma UK 31. Sarcoma follow up survey results Helen Stradling, Sarcoma UK 32. The Prevalence of skip metastases on whole bone MRI for high grade sarcomas of major long bones Ian Pressney, RNOH 33. The clinical and imaging differentiation of aneurysmal bone cyst and telangiectatic osteosarcoma Ian Pressney, RNOH

24. Scalp angiosarcoma: challenges of bolus application and comparison of two bolus techniques Gareth Ayre, Bristol Cancer Institute

34. Skeletal Staging of High-Grade Osteosarcoma: A Comparison of Bone Scintigraphy and Whole Body MRI Ian Pressney, RNOH 35. Pilot study to investigate the relationship between lipomatous lesions by MR texture analysis Ian Pressney, RNOH

25. Introduction of Treatment Summary at RJAH Geraint Davies, Robert Jones & Agnes Hunt Orthopaedic Hospital 26. Lorlatinib for the treatment of metastatic inflammatory myofibroblastic tumour: a case report Han Hsi Wong, Cambridge University Hospitals NHS Foundation Trust 27. Perivascular Epithelioid Cell Tumour

28. Primary Abdominal Wall Sarcomas: An experience of 57 patients surgically treated at two tertiary referral centres Hannah Tattersall, University of Birmingham

36. Chondral tumours: correlation between needle biopsy and surgical histology Ines Oliveira, RNOH 37. Myxoid liposarcoma – Unusual metastatic pattern and implications for staging and follow-up: A literature review

Page 22

Ines Oliveira, RNOH

Johanne Vass, Abertawe Bro Morgannwg University Health Board

38. Staging Investigations Employed in the Diagnosis of Chordomas Inga Usher, The National Hospital for Neurology and Neurosurgery

47. Patient preferences in orthopaedic oncology: are patients’ wishes for receiving diagnosis being met?

Jonathan Gregory, Hull York Medical School

39. What is a benign notochordal cell tumour? Clinical, radiological and histological characteristics from a large systematic review Inga Usher, The National Hospital for Neurology and Neurosurgery

48. Predictors of non-benign lipomas: evidence from a supraregional sarcoma service Karishma Khan, Cardiff University Medical School

40. Sarcoma quality performance indicators (QPIs) driving continuous improvements in Scotland Ioanna Nixon, NHS Glasgow and Clyde

49. The use of Dermal Substitute (Pelnac®) after sarcoma excision: a case series Lindsay A Shanks, Nottingham University Hospital NHS Trust

41. Pre-sacral lipomatous lesions – True liposarcomas or co-incidental curiosity?

Hannah Tattersall, University of Birmingham

50. Extra skeletal myxoid chondrosarcoma: a monocentric retrospective study.

Louis-Romee Le Nail, Royal Orthopaedic Hospital of Birmingham - Oncology department

42. Uterine Sarcoma: a single-centre’s experience of treatment and outcomes Ishan Mehta, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust

51. One year Snap-shot of Modern Operative Management of Soft-tissue Sarcomas: The Nottingham Experience Lucie Wright, Nottingham University Hospitals

43. Head and neck pleomorphic dermal sarcoma – lessons learned from 57 consecutive cases in South Wales James Warbrick-Smith, Welsh Centre for Burns and Plastic Surgery 44. Success of revision surgery for infected massive endoprostheses as identified from hospital coding James Grant, Royal National Orthopaedic Hospital 45. Extra-articular resection for Osteosarcoma – Are we getting it right?

James Barnett, Royal National Orthopaedic Hospital NHS Trust 46. “Engaging with adults living with soft tissue sarcoma to inform the development of a new supportive care service – what we learned”

52. Primary bone tumour patients’ experiences of symptoms prior to diagnosis: a systematic review and qualitative synthesis Lucinda Gosling, The Royal Orthopaedic Hospital NHS Trust 53. MRI of Extra-Skeletal Ewing Sarcoma – a 10 Year Experience Michael Khoo, Royal National Orthopaedic Hospital 54. A case of delayed response to Olaratumab and remarkable neurological recovery in metastatic soft tissue sarcoma Michael Rowe, Sunrise Oncology Centre, Royal Cornwall Hospital NHS Trust 55. An audit of re-staging chest CT at first local recurrence of soft tissue sarcoma

Page 23

Mirza Baig, Barts and the London School of Medicine and Dentistry

differentiated retroperitoneal liposarcoma (WD-RPLS) reveals recurrent mutations in FOXD4L3 gene Robert Tyler, Queen Elizabeth Hospital

56. Drug sensitivity testing of soft tissue sarcoma cells at surgery to select personalised chemotherapy: report of a pilot study Nicholas C Eastley, University of Leicester

65. Ten year follow- up of patients with soft tissue sarcoma; East Midlands experience Rossel Morhij, Nottingham University Hospitals

57. Preliminary Results of MRI Dixon technique for diagnosis of focal bone lesions Niels van Vucht, Royal National Orthopaedic Hospital

66. Metronomic cyclophosphamide and prednisolone in patients with pre-treated metastatic sarcoma: a single centre retrospective audit Ruhi Kanani, University College London Hospital

58. Primary Spindle Cell Sarcoma of Bone: Imaging Features in 37 Patients Asif Saifuddin, Royal National Orthopaedic Hospital NHS Trust, Stanmore

67. A survey to investigate current local practice in the UK for the testing of hormone receptors in ULMS /ESS and the impact this result has on whether HRT is considered Sam Hackett, Sarcoma UK

59. Retrospective Review of free fibula transfer in paediatric sarcoma: A single Centre Study of epiphyseal transfers Paul Roblin, Guy’s & St.Thomas Hospital, London, UK

68. Sarcoma Limb Immobilisation Samantha Farrington, MPCE - RTh Physics Singleton Hospital Swansea

60. Dosimetric characteristics, outcomes and prognostic factors of patients from University College London Hospitals (UCLH) with extracranial chordomas and chondrosarcomas treated with high dose radiotherapy Mahbubl Ahmed, University College London Hospitals

Thank you to our

Sherron Furtado, Royal National Orthopaedic Hospital, Stanmore, London, UK

Charity Supporters:

74. The route to diagnosis (RtD) of sarcoma patients: a qualitative study in the Netherlands and the United Kingdom V.L.M.N. Soomers, Radboudumc 75. The QUEST study: assessing the QUality of life and Experiences of Sarcoma Trajectories: study protocol V.L.M.N. Soomers, Radboudumc

Established in 2006, the Bone Cancer Research Trust is now the leading charity dedicated to fighting primary bone cancer.

Based in Leeds, the charity’s mission is to save lives and improve outcomes for people affected by primary bone cancer through research, information, awareness and support. www.bcrt.org.uk

76. SSGXXII - Three versus five years of adjuvant imatinib as treatment of patients with operable GIST with a high risk of recurrence: A randomised phase III multicentre study by the Scandinavian Sarcoma Group V Ramesh Bulusu, Cambridge University Hospitals NHS Foundation Trust 77. MS-275 inhibits sarcoma stress adaptation and enhances the effects of Doxorubicin and Cisplatin Xue Qi Wang, Dept of Molecular Oncology, BC Cancer Research Centre, Vancouver, Canada

69. Is it time for a national sarcoma database?

Sandra McAllister, Morriston Hospital 70. Why Having the Right Numbers Count Sarah McDonald, Sarcoma UK 71. Management of primary soft tissue spinal sarcomas at the Royal Marsden Sarcoma Unit 2000-2017 Dr Emma Burke, The Royal Marsden Hospital

61. Very Late Relapse In High Grade Soft Tissue Sarcoma In Extremities Qi Yin, Royal Liverpool University Hospital 62. What is the significance of a ‘whoops’ procedure? A review of the world literature Robert Grimer, Royal Orthopaedic Hospital, Birmingham 63. Two-stage surgery for myxofibrosarcoma: Resect & VAC Robert U. Ashford, East Midlands Sarcoma Service 64. Long read nanopore sequencing of well

72. Developing a holistic personalised model of rehabilitation care to optimise outcomes after treatment for lower extremity sarcoma Dr Sherron Furtado, Royal National Orthopaedic Hospital, Stanmore, London, UK.

Sarcoma UK is a national charity that funds vital research, offers support for anyone affected by sarcoma cancer and campaigns for better treatments. It is one of the biggest funders of sarcoma research in the UK and the only charity in the UK focusing on all types of sarcoma. sarcoma.org.uk

73. A markerless motion capture to quantify functional outcomes using a depth-sensor after treatment for lower extremity sarcomas in the clinic: A feasibility and validity study

Page 24

GIST Support UK provides support & information to patients with Gastrointestinal Stromal Tumours and to their families and friends As the only UK charity focusing solely on GIST we work to raise awareness & create an infrastructure that stimulates research to improve treatment & find a cure for GIST cancer. www.gistsupportuk.com www.pawsgistclinic.org.uk

Page 25

Thank you to our Sponsors Main Partner:

Immedica and Medical Need (part of Immedica Group) have a mission to be a partner and service provider that identifies and satisfies unmet medical needs of individual patients and/ or situations in health care, in close collaboration with research institutions, industrial partners, health care professionals, patient organizations and other key stakeholders.

Exhibitor Floorplan Main Conference room

Premium Sponsor:

4

5

1

www.immedica.com

3

2

For more than fifty years, Lilly has been dedicated to delivering life-changing medicines and support to people living with cancer and those who care for them. Lilly is determined to build on this heritage and continue making life better for all those affected by cancer around the world. www.lilly.co.uk

9

Supporting Sponsor:

At TAKEDA ONCOLOGY, we endeavour to deliver novel medicines to patients with cancer worldwide through our commitment to science, breakthrough innovation and passion for improving the lives of patients. This singular focus drives our aspirations to discover, develop and deliver breakthrough oncology therapies.

8

7

6

Exhibitors 1. Takeda

7. Sarcoma UK

2. Bayer

8. Champions Oncology

3. Immedica

9. Rutherford Clinic

www.takeda.co.uk

Thank you to our Exhibitors:

4. Immedica 5. Bone Cancer Research Trust 6. GIST

Page 26

Page 27

DAY ONE - SESSION: 08:55 - 09:45 Outcomes after hindquarter amputation

Mifamurtide significantly increased the overall survival of patients with newly-diagnosed resectable high-grade osteosarcoma when used in conjunction with combination chemotherapy when compared to chemotherapy alone.

MEPACT is indicated in children, adolescents and young adults for the treatment of high-grade resectable non-metastatic osteosarcoma after macroscopically complete surgical resection. It is used in combination with post-operative multi-agent chemotherapy. Safety and efficacy have been assessed in studies of patients 2 to 30 years of age at initial diagnosis.

Information about this product, including adverse reactions, precautions, contra-indications and method of use can be found at www.medicines.org.uk/emc. Please consult the Summary of Product Characteristics before prescribing.

Adverse events should be reported. Reporting forms and information can be found at www.mhra.gov.uk/yellowcard. Adverse events should also be reported to Takeda UK Ltd on 01628 537 900.

Takeda Oncology and

are registered trademarks of Takeda Pharmaceutical Company Limited.

Other trademarks are the property of their respective owners. © Takeda UK Limited, 2019. Legal category: POM. Takeda UK Limited, Building 3, Glory Park, Glory Park Avenue, Wooburn Green, Bucks, HP10 0DF is responsible for the sale and supply of Mepact in the UK. UK/MEP/1701/0001(2)

Date of Preparation: February 2019

and mean hospital stay duration was 48 days.

Christian Gray Stephens, RNOH Stanmore; Benjamin Davies, RNOH Stanmore; Andrew Johnston, RNOH Stanmore; Skinner J, Jagiello J, Pollock R, Aston W, Gikas P, Briggs T, Gunning P, Gerrand C. Introduction Hindquarter amputation is removal of the lower limb and part of the pelvis. It is a radical surgical procedure, most commonly performed for attempted cure or palliation of pelvic tumours when limb sparing surgery is not possible or for intractable infection.

Aim Our aim was to describe the experience of hindquarter amputation in our centre.

Specifically, the patient population, indications for surgery, complications and outcomes including local and systemic relapse and overall survival.

Methods This was a retrospective data analysis of patients treated since 1991 in our centre. Data was collected from medical documentation, imaging, histopathological reports, multidisciplinary correspondence and coding.

Primary outcome was all cause mortality and secondary outcome was disease recurrence.

Wound complications were recorded.

Results 154 patients underwent hindquarter amputation. 97 were male and 57 female of mean age 52 years (range 9-84). Common indications include chondrosarcoma (n=50) and osteosarcoma (n=20). 5 hindquarters were performed for infection. Overall survival was 25% at 5 years and 15% at 10 years (n=21 at 5 years and n=11 at 10 years). Lower grade tumours had more favourable survival at 5 years (Grade 1&2 = 77% and Grade 3 = 14%).

Wound complications were prevalent. Mean physiological burden of operation was high

Conclusion Despite high mortality and morbidity, hindquarter amputations are often a necessary procedure. The risks should be considered, and discussed with the patient, when considering hindquarter amputation.

Unfortunately, survival is poor. This reflects the aggressive nature of these tumours.

DAY ONE - SESSION: 08:55 - 09:45 Reconstructing lower limbs of the growing child with the Juvenile Tumour System

Christian Gray Stephens, RNOH Stanmore; Saleem Hussenbocus, RNOH Stanmore; Craig Gerrand, RNOH Stanmore; R Pollock1, A Johnston1, W Aston1, G Blunn2, T Briggs1, P Gikas1. RNOH Stanmore1, Institute of Orthopaedics and Musculoskeletal Science, UCL2 Introduction Limb salvage surgery is the mainstay of treatment for the management of bone sarcomas affecting the limbs due to advances in endoprosthetic replacements and adjuvant therapy. The Juvenile Tumour System (JTS) is useful in lower limb reconstruction in skeletally immature patients where the growth plate is affected. NIEE can be lengthened without anaesthesia and surgical incision, offering benefit over previously used minimally invasive growers.

Method Long term follow up of 98 children who have received JTS of the lower limb is presented.

Second operation was stratified by indication; loosening, infection, arthrosis, increase growth potential, implant failure and disease progression. Osseointegration and stem radiolucency was assessed.

Results Mean age of diagnosis was 10.5. 26/98 died

Page 29

Abstracts - Free Papers

Abstracts - Free Papers

Conclusion NIEE can be used to treat paediatric bone tumours of the lower limb with good functional outcomes. Osseointegration is seen but perhaps can be improved to reduce aseptic loosening. Further operations in this paediatric population remains a common occurrence.

DAY ONE - SESSION: 08:55 - 09:45 Inferior vena cava (IVC) leiomyosarcomas involving bilateral renal veins – is surgical resection without reconstruction safe?

Twenty-five patients with localized radiationinduced sarcoma of bone who received a definitive surgery were analysed. The definitive surgery was the limb salvage surgery in 15 patients and the amputation in 10. Fourteen patients received preoperative chemotherapy.

Results: Seven of ten patients were females (70%).

The IVC was resected en-bloc with the right kidney in all cases. In 9 cases the IVC was ligated without reconstruction, in one case only the IVC was reconstructed. In all cases the uninvolved left renal vein remained stapled in situ without vascular reconstruction. The renal function was abnormal immediately postoperatively, peaking on day 3 postoperatively but returned to normal values by day 12 for most patients (8/10). No patient required long-term renal dialysis.

Results: The 5-year overall survival rate (OS) and the 5-year event-free survival rate (EFS) were 53% and 40%, respectively. Patients with wide surgical margins (>2 mm) showed significantly better OS and EFS compared with those with narrow (0.1 to 2 mm) or involved margins (P = 0.033 and 0.002, respectively). Patients with preoperative chemotherapy showed significantly better metastatic-free survival (P = 0.040). The 5-year local recurrence-free survival was significantly better in the amputation group compared to that of the limb salvage group (100% vs 49%, P = 0.017). The rates of additional surgeries for complications were significantly higher in the limb salvage group (33% vs 0%, P = 0.041). OS was not significantly different between the limb salvage group and the amputation group (P = 0.188).

Conclusion: Ligation of the left renal vein without reconstruction is a safe option. Even combined with right nephrectomy, patients develop sufficient collaterals with only mild – moderate reduction in renal function.

DAY ONE - SESSION: 08:55 - 09:45 Surgical and oncological outcomes of localized radiation-induced sarcoma of bone

Dirk Strauss, Royal Marsden Hospital; Nikos Memos, Royal Marsden Hospital; Andrew Hayes, Royal Marsden Hospital; Myles Smith Background Sarcomas involving the IVC often require resection of a segment of the IVC. When the tumour involves the inter-renal segment of the IVC, surgical resection commonly necessitates a right nephrectomy. There is no data regarding the need for vascular reconstruction of the left renal venous drainage after resection of the inter-renal IVC. The aim of this report is to present our data on resections of the interrenal IVC segment with right nephrectomy without vascular reconstruction of the left renal vein.

Patients and Methods: From 2010 – 2016, 70 patients with retroperitoneal leiomyosarcoma had surgery, including ten patients who underwent

resection of the inter-renal segment of the IVC and right nephrectomy. The left renal vein was ligated and no reconstruction of the renal vein performed. Data collected includes clinicopathologic parameters, renal function, postoperative complications and survival data.

Yusuke Tsuda, Oncology department, Royal Orthopaedic Hospital; Martin Lowe, Oncology department, Royal Orthopaedic Hospital; Jonathan Stevenson, Oncology department, Royal Orthopaedic Hospital; Michael Parry Oncology department, Royal Orthopaedic Hospital; Lee Jeys, Oncology Department, Royal Orthopaedic Hospital Introduction: The aim of our study is to determine surgical or oncological outcomes and prognostic factors in localized radiation-induced sarcoma of bone. We also aimed to compare these outcomes between patients who received a limb salvage surgery and patients who received an amputation.

Material and Methods:

Page 30

Conclusions: We believe that a resection with wide resection margins and preoperative chemotherapy lead to better survival outcomes. Limb salvage surgeries are significantly associated with higher rates of local recurrence and postoperative complications compared with those of the amputation group.

DAY ONE - SESSION: 08:55 - 09:45 West of Scotland Breast Sarcoma Audit

Naomi Foster, Scottish Sarcoma Network; Fiona Cowie, Ioanna Nixon, Steven Lo, Jeff White, Lindsay Campbell, Scottish Sarcoma Network Background: Breast sarcoma is rare, <1% of breast cancers, and <5% of sarcomas. Early referral to a specialist centre may ensure the first procedure achieves clear margins which may translate into a survival advantage.

Methods:

Patients were identified from the West of Scotland Cancer Registry and pathology databases. Demographics, tumour and treatment details was recorded.

Results: 46 patients (Age 26-88) between 2006 - 2018.

Of the 46 patients, 24 had angiosarcoma, 12 spindle cell, 7 pleomorphic/undifferentiated, 2 liposarcoma, and 1 fibrosarcoma. Most were high grade (37/46). 29/46 had previous same side breast cancer, 21/29 had prior radiotherapy for this. 42 patients underwent surgery. 24 underwent initial surgery in a sarcoma centre, with 2 positive margins (8.3%).

18 cases were operated at peripheral hospitals, with 7 postive margins (39%). The difference in positive margins was statistically significant (p=0.025). Wide local excision was the first procedure in 7 patients, all performed at peripheral hospitals. No wide local excisions were performed at the sarcoma centre, with a more aggressive surgical approach including radical mastectomy in most cases, up to and including rib resection (1 case).

Conclusion: Higher rates of clear surgical margins are attained when the primary sarcoma resection is performed at a high-volume sarcoma centre. This may translate into an improved survival outcome. We advocate early referral to specialist sarcoma centres and intend to produce a Scottish Breast Sarcoma Guideline to facilitate this.

DAY ONE - SESSION: 14:00 - 15:00 Incidence and survival of soft tissue sarcoma in England between 2002 and 2016: an analysis from the National Cancer Registration and Analysis Service (NCRAS)

Andrew O Bacon, Public Health England; Sandra J Strauss, University College London and Public Health England; , Public Health England; John Broggio, Public Health England; Kwok F Wong, Public Health England Introduction Historically, survival analysis of soft tissue sarcoma (STS) has been limited to generalised

Page 31

Abstracts - Free Papers

with mean survival was 23 months. Mean follow up was 5 years (0-14.6). Common histopathological diagnoses included osteosarcoma (n=76) and Ewing’s sarcoma (19). Prostheses used include DFR (n=61), PTR (n=18) PFR (n=15) and TFR (n=4). Second operation (n=49) description was; 17 for lengthening potential, 8 chest metastasis excision, 6 for loosening, 5 for local tumour recurrence, 4 for arthrosis of the acetabulum, 3 for fracture, 3 for implant failure, 2 for infection and 1 for foot drop. Mean 1.33/4 sides of hydroxyapatite collar osseointegration occurred, and mean radiolucency was 2.84/24 around stem

Methods Patients diagnosed with sarcoma between 2002 and 2016 were extracted from the NCRAS database. Age-standardised incidence rates were reported per 1,000,000. Time at risk began at date of diagnosis and continued until 31st of December 2016. Five-year KaplanMeier survival was reported according to STS groupings. All tests were conducted using 5% level of significance.

Results The median age at diagnosis for STS was 63 years (IQR, 48-75). There are no significant changes in incidence over the 15-year period. Five-year survival varies by grade, ranging from 95.9% - 56.4% (MPNST; vascular) for grade 1, 65.2% - 41.3% (TUD; vascular) for grade 2 and 46.8% - 23.8% (liposarcoma; sarcoma NOS) for grade 3. Survival varied significantly according to the site of disease, with respiratory and intrathoracic organs amongst the poorest performers.

Conclusion This population-based analysis offers the most granular review of incidence and survival of STS to date. Grouping sarcomas into morphologically similar cohorts offers greater insight into the clinical and histological parameters that define outcome.

DAY ONE - SESSION: 14:00 - 15:00 The Breast Angiosarcoma Surveillance Study (BRASS)

Jenny Banks, Torbay and South Devon NHS Foundation Trust; Prof. C Holcombe, Royal Liverppol and Broadgreen University Hospitals NHS Trust; Prof. L Wyld, University of Sheffield;

Miss S Potter, University of Bristol

interventions.

Background Breast angiosarcomas (AS) are rare, poorly understood tumours which may develop spontaneously (primary AS, (PAS)) or secondary to lymphoedema or radiotherapy following breast cancer (secondary AS, (SAS)). Data on optimal management, prognosis and outcomes is scarce. This retrospective cohort study seeks to improve understanding of this disease and help inform a future prospective study.

Bass Hassan, University of Oxford; Gesa Tiemeier, University of Oxford; Ruth Macpherson, Oxford University Hospitals NHS Trust; Sarah Pratap, Oxford University Hospitals NHS Trust Shirley Henderson, Oxford University Hospitals NHS Trust, Ana Shuh, Oxford University Hospitals NHS Trust Nick Maynard, Oxford University Hospitals NHS Trust Robert Marshall, Oxford University Hospitals NHS Trust, Kevin M. Bradley, Oxford University Hospitals NHS Trust

Methods Breast, plastic surgery and sarcoma centres across the UK treating breast AS were invited to participate through trainee research collaborative networks. All patients with a tissue diagnosis of primary or secondary AS of the breast or chest wall between 2000 and 2015 were eligible for inclusion. Data regarding prior breast cancer, its treatment, AS histology, management and outcomes were collected.

Results To date, 61 patients have been entered from 10 centres. All patients were female and the majority presented with SAS (n=52, 85%). 85% of patients were considered to be resectable at the time of diagnosis; 46% of patients were managed primarily by breast surgeons, 31% by plastic surgeons and 8% by dedicated sarcoma surgeons. Twenty-three patients (38%) were treated within regional sarcoma centres. 62% (38) of patients were reported to have been discussed at a breast MDT and half were discussed at a specialist sarcoma MDT (n=32 52%).

Discussion Guidelines suggest these tumours should be managed within sarcoma centres, however this currently appears to be aspirational and regional variation is significant. Further data is required.

DAY ONE - SESSION: 14:00 - 15:00 Retrospective evaluation of 294 consecutive 18F-FDG PET-CT scans in gastro-intestinal stromal tumours (GIST) with respect to driver mutation subtypes and treatment

Page 32

Consecutive PET-CT scans (294) were performed in a single supra-regional centre in 183 GIST patients between 2007-2017.

Of the 183 patients, 103 had GIST driver mutation testing. We compared PET-CT SUVmax in relation to size of the tumour, mitosis per 50 high power fields and driver mutation subtype, SUVmax response over time according to imatinib dosing. SUVmax values ranged from background to >30 in GIST, with statistically significant differences in mean SUVmax between driver mutations. PDGFRA D842V mutation (mean SUVmax 2.3) and KIT exon 9 (SUVmax 5.4), had a significant lower SUV max at baseline compared to patients without mutations identified (mean SUVmax 13.4, p<0.05), and those with KIT exon 11-deletion (mean SUVmax 15.2, p<0.005), with the latter being higher than KIT exon 11 missense mutations (p<0.05). Unlike SUVmax, comparison of the either mitotic count, tumour location or size, grouped by driver mutation, revealed no significant correlations. Response to imatinib using SUVmax selected optimal imatinib dosing. Analysis of longitudinal associations using generalised estimated equations (GEE) of repeated SUV max measurement on imatinib treatment, revealed significant a difference in intercept (baseline SUVmax) between patients with a diver mutation and those deemed wild-type (wildtype SUVmax= 9.08, mutation positive GIST SUVmax= 8.1; p<0.05), with the regression coefficients of both groups being negative and similar (B=-0.386 vs B=-0.264, respectively; p>0.05). PET-CT in GIST may add significant benefit in GIST management as there are SUVmax associations with driver mutations.

We support further evaluation of PET-CT combined with mutation testing in GIST.

DAY ONE - SESSION: 14:00 - 15:00 Radiological and Histopathology alterations following pre-operative Radiotherapy in Soft Tissue Sarcomas of the extremities

Nikolaos Memos, The Royal Marsden NHS Foundation Trust; Christina Messiou, The Royal Marsden NHS Foundation Trust; Neil Soneji, The Royal Marsden NHS Foundation Trust; Anna-Maria Sharli, The Royal Marsden NHS Foundation Trust; Anisha Patel, The Royal Marsden NHS Foundation Trust, Dirk Strauss, The Royal Marsden NHS Foundation Trust; Myles Smith, The Royal Marsden NHS Foundation Trust; Shane Zaidi, The Royal Marsden NHS Foundation Trust; Eleanor Moskovic, The Royal Marsden NHS Foundation Trust; Andrew J Hayes, The Royal Marsden NHS Foundation Trust; Aisha Miah, The Royal Marsden NHS Foundation Trust Purpose/Objective: The purpose of the present study is the evaluation of the post-RT MRI features for Soft Tissue Sarcoma(STS) of the extremities.

Material/Methods: Data from patients with STS treated with preoperative RT and surgery for soft tissue sarcomas in our institution were retrospectively collected between 2007 and 2017. Crosssectional MRI imaging obtained before and 4 weeks after completion of RT. Data included demographics, tumour grade and histopathological subtype. MRI features documented were: tumour size (cm), contrast enhancement (%), cystic and haemorrhagic components (%) and the depth of peri-lesional oedema (in mm) on pre and post RT MRI. Follow up data was collected from patients’ records to assess overall survival (OS), local recurrence (LR) and distant metastasis (DM).

Results: A total of 167 patients who received preoperative radiotherapy were included. There was a significant decrease in the percentage contrast enhancement post-RT (p=0.001)

Page 33

Abstracts - Free Papers

anatomical groupings of soft tissue and gastrointestinal stromal tumours to compensate for the rarity of disease. Generic groupings of STS fail to offer clinically relevant insights into disease outcome. Morphologically similar STS were grouped according to WHO criteria to produce 13 cohorts (N>700) to allow for more detailed analysis of incidence and survival according to grade and the site of disease.

Conclusion: The MRI changes following radiotherapy vary with pathological subtype. Potential correlations with survival outcome require prospective evaluation.

DAY ONE - SESSION: 14:00 - 15:00 RNA sequencing of Ewing’s sarcoma patient-derived cancer stem-like cells identifies novel biomarkers of risk and putative targets for therapy

Dr Elizabeth Roundhill, Children’s Cancer Research Group, Leeds Institute of Medical Research, St. James’s University Hospital; Ms Mariona Chicon-Bosch, Children’s Cancer Research Group, Leeds Institute of Medical Research, St. James’s University Hospital; Mr Kenneth Rankin, Freeman Hospital; Mr Michael Parry, The Royal Orthopaedic Hospital; Professor Lee Jeys, The Royal Orthopaedic Hospital; Professor Susan Burchill, Children’s Cancer Research Group, Leeds Institute of Medical Research, St. James’s University Hospital Background Cancer stem-like cells (CSCs) are drivers of disease progression and relapse (Schatton et al. Bioessays, 2009.31:p1038-49). Therefore eradication of Ewing’s sarcoma (ES)-CSCs is expected to improve outcomes for some patients. Using functional assays we have isolated ES-CSCs from tumours and profiled paired parental ES and ES-CSC cultures to identify candidate drivers of risk and targets for treatment.

Methods ES and ES-CSCs were isolated from fresh tumour biopsies following patient consent (IRAS-167880). Cells were confirmed to be ES by expression of CD99 and the EWSR1ETS translocation. Total RNA libraries were sequenced (HiSeq3000, illumina®), reads aligned (STAR) and differential expression determined (DeSeq2). Candidate genes were validated using RTqPCR and immunocytology.

The prognostic significance of the top-hit was evaluated by immunohistochemistry of tumours, Kaplan-Meier curves and Cox Proportional Hazards Model.

Findings RNA sequencing revealed 6 candidate genes that were significantly enriched in ES-CSCs compared to paired parental cultures (adjusted p value<1x10-4, >3-fold increase, n=16).

Increased expression of candidate genes was confirmed by RTqPCR and immunocytology.

High expression of the top hit strongly predicted for event free survival (EFS, n=36, p=0.0078; Hazard Ratio=6 p=0.019). This was confirmed in an online dataset (GSE17618, n=34; p=0.0093; Hazard Ratio=3 p=0.012).

Furthermore, expression of two binding partners that regulate the expression of the top candidate also predicted EFS (p<0.017; Hazard Ratio=>2 p<0.05). Consistent with the hypothesis that this pathway drives progressive disease, knock-down of the candidate modulated self-renewal and drug resistance.

Conclusions RNA profiling of ES-CSCs identifies novel prognostic biomarkers and putative targets for therapy.

DAY ONE - SESSION: 14:00 - 15:00 A Phase 1 Study Of The Poly-Adp Ribose Polymerase (PARP) Inhibitor, Niraparib (NIR), In Combination With Irinotecan (IRN) In Patients With Advanced Ewing Sarcoma: Results Of Sarc025 Arm 2

Rashmi Chugh, University of Michigan; Sandra Strauss, University College London Hospitals NHS Trust; Karla Ballman, Weill Cornell Medicine; Lee Helman, Children’s Hospital

Page 34

of Los Angeles; Douglas Hawkins, Seattle Children’s Hospital; Denise Reinke, Sarcoma Alliance for Research through Collaboration

DAY TWO - SESSION: 10:50 - 11:50 Desmoid fibromatosis through the patients’ eyes: time to change the focus and organisation of care?

Objective: To determine the dose-limiting toxicities (DLT) and maximum tolerated dose of NIR in combination with IRN in patients with pretreated ES. Methods: Using a 3+3 design, eligible patients > 13 years with recurrent/relapsed ES were assigned to cohorts evaluating NIR 100-300mg od D1-7 and IRN 20-50mg/m2 D2-6 (28 day cycle) commencing at 100mg NIR and 25mg/m2 IRN (Dose level 1). Pre-treatment tumour biopsy was mandated for patients with accessible disease and post-treatment for patients > 18 years.

Results: Twelve eligible patients were enrolled at 2 dose levels. Median age was 27 years (range 16-50); median prior therapies 4 (range 1-2); 6 patients received prior IRN. Patients received a median of 2 cycles (range 1 - 17). DLTs were observed in all 3 patients at dose level 1 (1 pt each with grade 3 anorexia, colitis and transaminitis). No DLTs were reported in 9 patients at NIR 100mg and IRN 20mg/m2 (dose level -1); 2 patients experienced transient grade 3 neutropenia and thrombocytopenia and 1 patient grade 3 gastro-intestinal toxicity. Best response was PR in 1 patient, SD in 6 patients, and PD in 5 patients. Median progression-free survival was 3.8 months (1.18-NR). Pharmacodynamic analysis of tumour samples demonstrated > 80% PARP inhibition.

Conclusions: NIR 100 mg qd D1-7 in combination with IRN 20mg/m2 D2-6 was well tolerated with preliminary evidence of efficacy that warrants further investigation. Patient biopsy was feasible and pharmacodynamic analysis supported ongoing study design. Further cohorts incorporating temozolomide are planned and additional correlative analysis is ongoing.

Olga Husson, Institute of Cancer Research; Eugenie Younger, Royal Marsden Hospital; Alison Dunlop, Royal Marsden Hospital; Lucy Dean, Royal Marsden Hospital; Robin Jones, Royal Marsden Hospital; Winette van der Graaf, Institute of Cancer Research; Dirk Strauss; Charlotte Benson; Andrew Hayes; Aisha Miah; Winan van Houdt; Shane Zaidi; Myles Smith; John Williams, Royal Marsden Hospital PURPOSE: Desmoid fibromatosis (DF) is a rare, unpredictable disease with no established, evidence-based treatments. Individual management is based on consensus algorithms. This study aimed to examine the specific health-related quality of life challenges faced by DF patients, current experiences and expectations of care.

METHODS: Twenty-seven DF patients were purposively sampled from The Royal Marsden Hospital.

Two focus groups and 13 interviews (males 12, females 15; mean age at study 39.5 years) explored health-related quality of life issues and experiences of healthcare. Thematic content was analysed.

RESULTS: Discussions revealed four key themes (diagnostic pathway; treatment pathway; living with DF; supportive care). Diagnostic delay resulted from lack of recognition by patients and healthcare professionals. Some patients received an initial diagnosis of cancer, causing significant distress. Treatment decisions were challenging, and patients experienced uncertainty among clinicians about optimal therapies. Side-effects of treatment were severe, including fatigue, nausea, anorexia, low libido and depression. Pain was the most debilitating symptom and dependency on painkillers was a significant concern.

Functional limitation and restricted mobility frequently affected daily activities. Patients experienced difficulty accomplishing their role

Page 35

Abstracts - Free Papers

and a significant increase in post-RT cystic component and peri-lesional oedema (p<0.001, p<0.001). Cystic component changes were more evident in pleomorphic sarcomas (p=0.023) and reductions in size in myxoid liposarcomas (p<0.001). The overall 3 year LR, DM free survival and OS were 85.8%, 59.8% and 77.4% respectively. In a multivariate Cox regression analysis, the depth of preoperative perilesional oedema was negatively associated with DM (HR:1.6 95%CI 1.18-2.2, p=0.002) and OS(HR:1.8 95%CI 1.2-2.5, p=0.002).

CONCLUSIONS: DF patients face complex physical, psychological and practical challenges.

Comprehensive care services are needed.

Increasing awareness may help to improve diagnostic pathways and overall patient experience.

DAY TWO - SESSION: 10:50 - 11:50 Real-world experience with doxorubicin and olaratumab in soft tissue sarcomas across South East England

Spyridon Gennatas, Royal Marsden Hospital NHS Trust; Thomas Carter, University College Hospital London; Peter Simmonds, University Hospital Southampton; Palma Dileo, University College Hospital London; Charlotte Benson, Royal Marsden Hospital NHS Trust Background Phase 2 data have shown the addition of olaratumab to doxorubicin significantly increases overall survival (OS) in patients with advanced soft tissue sarcomas (STS) compared to doxorubicin alone.

Methods We performed a retrospective analysis of 105 patients treated with doxorubicin and olaratumab at three sarcoma units in the South East between May 2017- October 2018. Inclusion criteria: >18 years old; locally advanced/metastatic STS; > 4 infusions of olaratumab and ECOG performance status (PS) 0-2.

Results A total of 104 patients were evaluable. The female: male ratio was 1.56. Leiomyosarcoma was the most common histological subtype (48 patients, 46%), followed by liposarcomas

(15, 14%). The mean number of doxorubicin cycles was 4.55 (2-6) and olaratumab infusions 10.70 (4-36). Of 104 patients, 22% had a partial response (no CR), 50% had stable and 28% progressive disease. The median progression-free survival (PFS) was 6 months. Twenty-four patients had died at the time of analysis. Fifty-four patients (51%) experienced neutropenia of any grade and 16 (15%) had febrile neutropenia. At least grade 1 anaemia was seen in 94 (90%). Grade 3-4 non-haematological toxicities were seen in 26 patients (25%). A clinically significant drop in left ventricular ejection fraction was seen in 4 (3.8%). Thirty patients (29%) required a dosereduction.

Discussion Preliminary results from this real-world experience closely resemble the so far published data (ANNOUNCE). At the time of submission 78% of patients are alive and OS is pending. Data from another four centres are being analysed and results will be updated.

DAY TWO - SESSION: 10:50 - 11:50 Clinical relevance of Immune checkpoints and HLA expression in chondrosarcoma

Dr Joseph Schwab, Harvard Medical School; Ahmad Al-sukaini, University of Birmingham; Sjoerd Nota, Harvard Medical School; Soldano Ferrone, Harvard Medical School Purpose: The aim of this study was to analyze the role of immunological events in chondrosarcoma.

To this end, the expression of immunologically relevant molecules such as HLA class I, PD-L1, B7-H3 as well as the extent of tumor infiltrating immune cells were correlated with the histopathological characteristics of the tumors and with the clinical course of the disease.

Experimental Design: A tissue micro-array containing 76 chondrosarcoma tumors were analyzed for (a) tumor infiltrating immune cells, (b) HLA class I and HLA class II expression and (c) immune check-points PD-L1 and B7-H3 expression following immunohistochemical staining

Page 36

with monoclonal antibodies. The results were correlated with tumor grade and survival.

Results: CD8+ immune cells were present in 85% of dedifferentiated tumors and in 20% of conventional tumors. HLA-A and HLA-B, -C expressions were defective in 15 and 0% of dedifferentiated chondrosarcoma and in 92 and 77% of conventional chondrosarcoma, respectively. In the latter group, HLA class I expression positively correlated with tumor grade. PD-L1 was expressed in 33% of the dedifferentiated tumors and was associated with shorter time to metastasis. B7-H3 was expressed in 66 and 88% of the conventional and dedifferentiated tumors, respectively.

In conventional chondrosarcoma, B7-H3 expression correlated with higher tumor grade and worse survival.

Conclusions: High grade and dedifferentiated chondrosarcoma are immunogenic and express Immune checkpoint, such PD-1/PDL-1 and B7-H3 that may allow them to evade the host’s immune response. This study provides a rationale to target B7-H3 and/or PD-1/PD-L1 in chondrosarcoma DAY TWO - SESSION: 10:50 - 11:50 Outcome of Patients with Localized High-Grade Osteosarcoma Treated with Mifamurtide

Kim Tsoi, Royal Orthopaedic Hospital, Birmingham; Michael Parry, Royal Orthopaedic Hospital, Birmingham; David Peake, Queen Elizabeth Hospital, Birmingham; Yusuke Tsuda, Royal Orthopaedic Hospital, Birmingham; Robert Grimer, Royal Orthopaedic Hospital, Birmingham; Sarah Coldicott, Queen Elizabeth Hospital, Birmingham Background: Mifamurtide is the only new agent to be approved for use in osteosarcoma in the last two decades. This drug is an immunemodulator that induces macrophage activation and has been shown to improve overall survival in non-metastatic high-grade osteosarcoma following complete resection of

the primary tumour. Experience with standard chemotherapy plus mifamurtide has not been reported since the North American randomized control trial was published ten years ago. The objective of this study was to review outcomes in British patients treated with this combination therapy.

Methods: All patients treated at the Royal Orthopaedic Hospital (Birmingham, UK) between 2010 and 2016 were reviewed using a prospectively collected database. Fifty-four osteosarcoma patients that received mifamurtide were identified and their demographics and outcomes analyzed.

Results: The average age at diagnosis was 16 years (range 5 to 36) and 37% of tumours occurred in the femur. Limb salvage surgery was performed in 85% of patients and more than half had 90% or greater chemotherapy-induced necrosis in their resection specimen. Average follow-up was 52 months (range 14 to 93). Five year overall and event-free survival was 85% and 70%, respectively. Seven patients died due to sarcoma and one from an unrelated primary at an average of 38 months (range 22 to 78) post-diagnosis. Metastases occurred in 24% of patients, most commonly localized to the lung (n=12/13). Mifamurtide was well tolerated with few toxicities recorded.

Conclusion: Mifamurtide is a safe and effective immunemodulatory agent in the treatment of localized high-grade osteosarcoma in the British population.

DAY TWO - SESSION: 10:50 - 11:50 High-dose chemotherapy for the treatment of recurrent and primary-refractory Ewing Sarcoma

Dr Rachael Windsor, University College London Hospitals NHS Foundation Trust; Dr Anthony Hamilton, University College London Hospitals NHS Foundation Trust; Dr Palma Dileo, University College London Hospitals NHS Foundation Trust; Dr Maria Michelagnoli, Dr

Page 37

Abstracts - Free Papers

in society; relationship problems, caring for children, employment and financial difficulties.

Social isolation and lack of understanding were common. The psychological impact of this ‘life-changing and life-long’ condition was profound. All patients requested knowledgeable healthcare professionals, more information, continuity of care and peer support.

DAY TWO - SESSION: 10:50 - 11:50 Tumour necrosis is significantly associated with reduced overall survival after curative resection of GIST

Objective: The prognosis for patients with recurrent or primary-refractory Ewing Sarcoma (RRES) is poor with 5-year post-relapse survival (PRS) rates of 10-19%. The role of High-dose chemotherapy (HDT) is conflicting. We aimed to review the outcome of all patients treated for RRES at a single centre.

Robert Tyler, Queen Elizabeth Hospital; Mr. Dominic Tan, Birmingham University Medical School; Miss. Dayna Arthur, Birmingham University Medical School; Mr. Max Almond, Consultant Sarcoma Surgeon, Queen Elizabeth Hospital, Birmingham; Mr. Samuel Ford, Consultant Sarcoma Surgeon, Queen Elizabeth Hospital, Birmingham; Mr. Anant Desai, Consultant Sarcoma Surgeon, Queen Elizabeth Hospital, Birmingham

Methods: 209 patients with RRES from 1995-2017 were included. Patient, disease, and treatment characteristics at primary diagnosis and relapse/progression were obtained from hospital databases and patient files.

Results: Median and 5-year PRS for all patients was 12.9 months (range 0.3-318.8) and 21.7% (standard error 3.2%), respectively. Superior PRS was observed in the HDT compared to non-HDT patients; 5-year PRS 52.5% vs 5.4% (LogRank P<0.0005) (HR 0.336 (95% CI 0.2010.562; P<0.0005). Multivariate analysis (MVA) identified relapse ?24 months (HR 1.626; 95% CI 1.032-2.562; P=0.036), extra-pulmonary metastasis at diagnosis (HR 1.869; 95% CI 1.196-2.922; P=0.006), extra-pulmonary relapse (HR 2.242; 95%CI 1.371-3.667; P=0.001), and combined local and pulmonary relapse (HR 2.656; 95% CI 1.379-5.115; P=0.003) as statistically significant negative prognostic factors affecting PRS. We developed a prognostic score using the MVA. This identified three groups (low-, moderate-, and high-risk) with significantly different PRS. 5-year PRS was significantly longer in low- and moderate-risk HDT patients.

Conclusion: HDT may improve PRS for patients with recurrent or refractory ES. The development of a prognostic score for patients at the time of relapse or progression allowed further analysis of the benefits of HDT amongst patients of different risk and demonstrated that the benefit is seen in low- and moderate-risk patients.

Introduction Tumour necrosis is associated with poorer outcomes in several malignancies, however in GIST the literature is inconclusive. We aimed to determine whether tumour necrosis was significantly associated with survival in resectable GISTs. Materials and Methods We retrospectively analysed a database of curative GIST resections performed from 20062017 in a specialist sarcoma unit. The database comprised open and laparoscopic resections.

Tumour necrosis, location, histology (mixed, spindle, epithelioid), genetic mutation, codon and age were analysed. Overall survival was estimated with the Kaplan-Meier method, and associations with prognosis were assessed using Cox regression models.

Results A total of 148 patients (55% Male) with a median age of 66 years (IQR: 49-83) were eligible for analysis. Of these, 63% (92) of patients had a gastric GIST, 18% (27) small bowel, 12% (17) duodenal and 7% (10) rectal.

Tumour necrosis was recorded on the final pathology report in 24% of patients (36/148).

Open surgery (HR 2.132, p=0.046), a mixed histological diagnosis (HR 3.395, p=0.001) and tumour necrosis (HR 3.167, p=0.001) were associated with significantly shorter survival.

Estimated five year overall survival rates were 60% vs. 83% in those with vs. without tumour necrosis, with subgroup analysis by tumour

Page 38

location finding similar results in gastric and non-gastric GISTS. Conclusion This work adds to a growing body of evidence that tumour necrosis should be added as a valuable prognostic factor to the current NIH classification. Multivariable analysis is now underway to determine if tumour necrosis is an independent predictor of unfavourable overall survival in GIST. DAY TWO - SESSION: 11:50 - 12:50 Diagnostic accuracy of MRI and histology in assessment of deep lipomatous tumours

Lydia Cairncross, Royal Marsden NHS Foundation Trust; Hayden Snow, Royal Marsden NHS Foundation Trust; Olivia Sjokvist, Royal Marsden NHS Foundation Trust; Myles Smith, Dirk Strauss, Andrew Hayes, Royal Marsden NHS Foundation Trust Introduction: Deep lipomatous tumours represent a large proportion of referrals to tertiary sarcoma centres due to the perceived risk of malignancy. The distinction between lipomas and atypical lipomatous tumours (ALT) is often subtle and MDM2-genetic amplification studies have become gold-standard in differentiation.

This study aims to assess for clinical differences between lipoma and ALT, and report the radiological and pathological accuracy in diagnosing these tumours.

Methods: A single-institution, retrospective case series was reported for all patients who underwent surgery at a tertiary-referral sarcoma centre for deep lipomatous tumours. All patients had a preoperative MRI scan and all tumours were assessed either pre- or postoperatively with MDM2-genetic analysis.

Results: 248 patients were included and, of these, 153 underwent surgery without a preoperative biopsy, based on MRI findings alone. Most tumours (67.3%) were benign lipomas on final analysis. Age, tumour size and location in the lower limb were independently associated with

a diagnosis of ALT on multivariable analysis.

Preoperative MRI scan had a high sensitivity for diagnosing ALT (90.1%), but tended to overestimate the diagnosis, with a specificity of only 51.5%. For tumours located in sites other than the lower limb, the negative predictive value of MRI was very high (95.5%).

Discussion: Despite concerns for malignancy, most deep lipomatous tumours are benign. MRI is very good for excluding ALT, especially in the trunk and upper limb where almost all tumours reported as lipomas were confirmed benign on MDM2 analysis. This can help to risk-stratify referrals to sarcoma centres, based on the site and MRI features.

DAY TWO - SESSION: 11:50 - 12:50 A daily diagnostic multidisciplinary meeting to reduce time to definitive diagnosis in the context of primary bone and soft tissue sarcomas

Laura Hartley, University of Birmingham; Mr Scott Evans, The Royal Orthopaedic Hospital, Birmingham; Mr Jonathan Gregory, The Royal Orthopaedic Hospital, Birmingham; Dr M Davies, A Sharif, S Hopkins, S Kelly The Royal Orthopaedic Hospital, Birmingham Introduction: One of the key recommendations from the 2016 BSG guidance is that management of primary soft tissue and bone sarcomas be rationalised to form peer-reviewed regional soft tissue sarcoma services and a smaller number of centres specialised in treating primary bone sarcomas. The specialist unit at the Royal Orthopaedic Hospital currently receives 150 to 200 referrals per month from GPs and hospital-based consultants. In order to cope with the increasing workload, the unit has introduced a daily diagnostic multidisciplinary meeting (DMDT) to help better direct resources, cut unnecessary spending and improve consistency in decision making Aims: To assess whether this intervention has improved the pathway, with the primary endpoint being a reduction in time to definitive

Page 39

Abstracts - Free Papers

Beatrice Seddon, Dr Sandra Strauss, Professor Jeremy Whelan, University College London Hospitals NHS Foundation Trust

Methods: Retrospective access to patient notes via the Onkos database and IMPAX imaging over a 1-month period in 2015 and 2018 Results: the DMDT demonstrated a reduction in TTDD of 9 days, an improvement in service efficiency with subsequent reduction in time to make the first management decision of 8 days. The service also has an added benefit in reducing patient miles travelled over the course of diagnosis by 24.41 miles. Heterogeneity analysis is ongoing Conclusion: Our audit found an improvement in the patient pathway and a reduction in TTDD with the introduction of a daily DMDT. Further analysis is needed to determine the clinical significance of this.

DAY TWO - SESSION: 11:50 - 12:50 A Rational Approach to Two Week Wait Referrals

Damien McDermott, Royal Derby Hospital; Kaye Rowley, Royal Derby Hospital; Joanne Douglas, Royal Derby Hospital; Mojca Persic, Royal Derby Hospital From its inception the two week wait system has experienced increasing demand that has imposed a matching burden upon the specialised sarcoma centres. As a consequence the specialist centres now receive a considerable number of potentially inappropriate referrals. In Derby, a substantial DGH, we run a dedicated weekly two week wait clinic. GPs in the locality have access via Choose and Book. In addition appropriate GP urgent none two week referrals and urgent referrals by other medical and surgical teams are also reviewed. The clinic is run by an orthopaedic surgeon supported by clinical nurse specialists.

There is radiology support with same day ultrasound scan and urgent MRI/CT performed where appropriate. Blood testing is also undertaken. There is administrative support and ongoing audit of compliance with targets

and review of imaging. Patients are triaged for referral to sarcoma services, either The Royal Orthopaedic Hospital Birmingham or East Midlands Sarcoma Service, Nottingham, the latter giving close support through their MDT to which we video link. Other patients are referred to local none sarcoma MDTs where likely or definite other malignancy is recognised or the referral downgraded from a two week pathway if benign problems are identified. In a four year period we have seen 676 adult patients including 545 two week waits. We have referred 116 patients on to sarcoma services for evaluation. Twenty seven sarcomas were identified. Two week wait compliance is 97.75% against the national target of 93%.

DAY TWO - SESSION: 11:50 - 12:50 Evaluation of telephone clinic service for 2-week rule referrals for suspected soft tissue sarcomas in a tertiary centre.

Nikolaos Memos, The Royal Marsden Hospital NHS Foundation Trust; Chevette March, The Royal Marsden Hospital NHS Foundation Trust; Alison Dunlop, The Royal Marsden Hospital NHS Foundation Trust; Dirk Strauss The Royal Marsden Hospital NHS Foundation Trust, Winan van Houdt, Antoni van Leeuwenhoek Hospital Amsterdam; Andrew J Hayes, The Royal Marsden Hospital NHS Foundation Trust; Myles Smith, The Royal Marsden Hospital NHS Foundation Trust Introduction: The volume of 2-week rule referral pathway (2WR) patients at tertiary sarcoma centres has significantly increased since its inception, which is challenging for specialized clinics to manage.

In response, we developed a virtual follow up clinic for patients referred under the 2WR for suspected soft tissue sarcoma, and present our experience to date.

Materials and Methods: From March 2016 to September 2016, the 2WR referrals were assessed by a Consultant Surgeon, and when there was a low suspicion for malignancy, further follow up was arranged in a telephone clinic (TC). This clinic was scheduled weekly, staffed by a clinical fellow

Page 40

with clerical support with a maximum of 10-15 appointments. The efficacy outcome of the TC measured, was the impact on waiting times for follow-up clinics, before and after the commencement of TC. Results: A total of 522 patients were referred under the 2WR during the study period. One hundred-seventy-nine (34.9%) were booked for follow-up at the TC. In total, 83.9% (N=149) of these patients were discharged at TC, as investigation confirmed a benign lesion or normal variant anatomy. The proportion of the patients waiting >60 min at our Sarcoma follow-up clinics was reduced from 38.9% to 13.4% during the study period, with an increase in the proportion of waiting < 30mins to be seen (73% vs. 61%) (p<0.0001).

Conclusion: The introduction of a virtual clinic in specialized centres had a significant beneficial impact upon the follow up clinic, improving efficacy of follow up and patient experience.

DAY TWO - SESSION: 11:50 - 12:50 Measuring what matters: developing the content of the Sarcoma Assessment Measure (SAM)

Ana Martins, University College London Hospitals; Rachel Taylor, University College London Hospitals; Jeremy Whelan, University College London Hospitals; Lindsey Bennister, University College London Hospitals; Lorna A Fern, National Cancer Research Institute; Craig Gerrand, The Royal National Orthopaedic Hospital; Maria Onasanya, University College London Hospitals; Lesley Storey, Queens University Belfast; Mary Wells, Imperial College Healthcare NHS Trust; Rachael Windsor, University College London Hospitals; Julie Woodford, The Royal National Orthopaedic Hospital Background: Introducing patient-reported outcome measures (PROMs) into clinical practice is known to improve patient-clinician communication, patient experience and outcomes. While there are many generic cancer

PROMs there are none developed for sarcoma so these may not capture issues that are tumour-specific. This paper will report how the content of the Sarcoma Assessment Measure (SAM) was identified to reflect the issues patients with sarcoma face when living with and beyond diagnosis.

Method and results: The content of SAM has been developed systematically over a number of stages: Indepth interviews were conducted with 121 patients: 50% male; aged 13-82; with STS (62%), bone (28%) and GIST (10%).

Content analysis of the interview transcripts identified 1,398 post-diagnosis experience statements Experience statements were reviewed, repetition was removed and sentences were refined to form 395 ‘items’ which were included in an Item Reduction Questionnaire (IRQ) grouped according to the quality of life domains of physical, emotional and social wellbeing, and financial wellbeing and sexuality. The IRQ was completed by 250 patients: 51% male; aged 17-89; with STS (62%), bone (37%) and GIST (<1%), who rated each item on importance/worry. Items with a mean score above 5 were initially retained for inclusion.

Conclusion: We have identified 66 key issues that are important to patient experience that are not reflected in other PROMS. This systematic process of using patient experience to develop the content of SAM will ensure it will measure what is important to patients.

DAY TWO - SESSION: 11:50 - 12:50 A qualitative exploration of the experiences of teenagers and young adults with sarcoma

Ana Martins, University College London Hospitals; Rachel Taylor, University College London Hospitals; Jeremy Whelan, University College London Hospitals; Lindsey Bennister, University College London Hospitals; Lorna A Fern, National Cancer Research Institute; Craig Gerrand, The Royal National Orthopaedic Hospital; Maria Onasanya, University College London Hospitals; Lesley Storey, Queens

Page 41

Abstracts - Free Papers

diagnosis (TTDD). Secondary endpoints included measurements of the efficiency of the service and whether there has been a reduction in heterogeneity in decision making

protective/risk factors identified could guide future interventions.

Introduction and Aims: Treatment of sarcoma often involves longterm hospitalisation, extensive surgery, loss of mobility, complex rehabilitation programmes.

Subsequently, poorer patient-reported outcomes are recorded in comparison to patients with other cancer types. Studies examining psychosocial functioning of those affected by sarcoma are needed to improve wellbeing. This study aims to explore the experiences of teenagers and young adults (TYA) with sarcoma.

Methods: Qualitative study design using semi-structured telephone/face-to-face interviews. A total of 35 AYA diagnosed with soft tissue and bone sarcoma (19 female) aged 13-39 years (M=28.8; SD=6.7) participated (25 diagnosed within 4 years; 4 had an amputation). Interviews were recorded and transcribed verbatim. Framework analysis was used to analyse the data.

Results: The health-related quality of life domains of physical, emotional and social wellbeing and healthcare professionals’ role were the overarching themes of analysis. Family, friends and interactions with peers with cancer were core elements of support. Patients had concerns about the impact of sarcoma on employment/school, fertility and sexuality.

Adaptation after treatment was influenced by the way TYA dealt with stress and adversity, with some struggling with finding their ‘new normal’. Rehabilitation and therapy/counselling services had a considerable role in TYA’s physical and emotional wellbeing; however access to these services was not equitable.

Discussion and Conclusions: A sarcoma diagnosis disrupts normality and initiates a process of re-examining expectations for self, daily life, and future hopes and plans.

This study showed the impact of sarcoma on TYA’s wellbeing and identity development; Page 42

Abstracts - Posters 1. Audit of post op orthopaedic oncology referrals – are outpatient appointments booked within the requested time frame?

Abby McCarthy, RNOH Stanmore Aim: Objectively assess how many patients referred to onward physiotherapy services are seen within the timescale requested. Background: Increasing number of patients not receiving timely physiotherapy from outpatient services following discharge leading to - Adhoc review by physiotherapy during consultant clinics - Absorption into outpatient caseload to bridge the gap - Time spent chasing referrals - Dissatisfaction from patients and clinicians due to compromised clinical outcome Methods: Prospective audit May to June 2018. Therapists noted for each patient -date of discharge, date referral sent, time span within which the appointment was requested and which team was referred to. Referring hospitals were later contacted to establish the date of initial assessments. Standard 1 Patients have been referred to an identified outpatient or community physiotherapy service before discharge from RNOH surgical wards 95% Standard 2 Patients receive follow up outpatient/community physiotherapy within the timeframe stated on the referral form 90% Results: 24 cases recorded. Complete data established for 14 referrals, 10 sets of data unavailable.

Standard 1 - 67% compliance Standard 2 42% compliance Conclusion: Consider the following with relation to practice - Timely discharge reports are imperative - Review rehabilitation pathway for those undergoing chemotherapy – 4 people UTA/ DNA their outpatient appointment

- Empowerment of people to follow up referrals - Does timing of input matter or does outcome level off after a period of time?

- Empowerment of people to continue therapy programme independently between inpatient discharge and outpatient follow up.

2. The Aesthetic approach to anterior abdominal wall tumours

Ahmed Emam (FRCSPlast); Giulia Colavitti (FEBOPRAS); Stephen Ali (MRCS); Thomas Chapman (FRCSPlast); Thomas Wright (FRCSPlast); Paul Wilson (FRCSPlast) Department of Plastic Surgery, Southmead Hospital, North Bristol NHS Trust, UK Introduction: Abdominal wall sarcomas are a heterogeneous group of rare malignant neoplastic conditions with different morphological patterns and originating from mesenchymal tissues. Soft tissue sarcomas are more prevalent in the extremities, but less common in the abdominal wall, while Desmoid fibromatosis is a rare but locally aggressive tumour. Desmoids do not metastasize but can cause significant morbidity and mortality by local invasion. These tumours may occur anywhere, but are commonly found on the abdominal wall and even the intestinal mesentery. Management of such abdominal wall tumours should be within a specialist multi-disciplinary team to ensure safe resection and robust reconstruction. We present the North Bristol NHS trust approach for management of abdominal wall tumours to achieve safe oncological clearance but also ensure acceptable cosmesis Methods: Retrospective analysis of data collected from a prospectively maintained excel sheets database during the period from 2012 to 2018. Hospital electronic records were reviewed to collect the histology, reconstruction, wound complication and recurrence rate

Page 43

Abstracts - Posters

University Belfast; Mary Wells, Imperial College Healthcare NHS Trust; Rachael Windsor, University College London Hospitals; Julie Woodford, The Royal National Orthopaedic Hospital

Discussion: The abdominoplasty incision and flap can provide superior cosmetic results over direct incision for management of anterior abdominal wall tumours. It places the suture line away from the reconstruction/implant site and hence provides safe coverage and robust wound closure. It may also avoid placing the incision line in the field of pre-operative radiotherapy.

3. Diagnostic accuracy and safety of CT guided biopsy of bone and soft tissue lesions performed under general anaesthetic (GA): a single centre experience on 454 patients

Alessandro Vidoni, Royal National Orthopaedic Hospital NHS Trust; Asif Saifuddin, Royal National Orthopaedic Hospital NHS Trust Purpose: To evaluate the effectiveness of CT-guided biopsy in determining the histological diagnosis of bone and soft tissue lesions.

Methods: We retrospectively reviewed 454 consecutive CT-guided biopsies under general anaesthesia (GA) for a suspected sarcoma performed in our institution between October 2016 and October 2018. All the procedures were performed either by an MSK radiologist with 22 years of experience (n=341) or by a fellow under his direct supervision (n=113). The samples were examined by a sarcoma pathologist. The safety of the procedure was also evaluated in terms of immediate post-procedural complications.

Results: The study included 454 patients (226 F; 228 M; mean age 41.1 years; age range 1-89 years). In

376 cases (83%) it was possible to determine the histologic subtype of the lesion. Of these, 228 were malignant lesions and 148 benign and these biopsies were considered as true positives). In 78 cases (17%) samples were considered non-diagnostic. Repeat biopsy revealed the presence of a tumour in 34 cases (7.5%) of which 19 (4.2%) were malignant (biopsies considered false negatives), while no tumour was identified in 44 (10%) (biopsies considered true negatives). No false positives were demonstrated in the cohort. Two immediate complications were recorded (0.4%). In one case arterial bleeding which was resolved with compressive dressing, and secondly a small asymptomatic pneumothorax which required no active management.

Conclusion: CT-guided biopsy under GA for bone and soft tissue lesions is a safe procedure with a diagnostic accuracy of approximately 93%.

4. Value of staging in non-conventional appendicular osteosarcoma.

Alessandro Vidoni, Royal National Orthopaedic Hospital NHS Trust; Muhammad Bushara, Royal National Orthopaedic Hospital NHS Trust; Veronica Attard, Royal National Orthopaedic Hospital NHS Trust; Asif Saifuddin, Royal National Orthopaedic Hospital NHS Trust Objective: To determine the prevalence of chest and skeletal metastases in patients with primary low-grade central osteosarcoma (LGCOS), parosteal osteosarcoma (POS) and periosteal osteosarcoma (PeOS) of the appendicular skeleton.

Materials and Methods: Retrospective review of all patients diagnosed with LGCOS, POS and PeOS between January 2007 and December 2017. Data collected included age, sex, and prevalence of skeletal metastases on BSc/WBMRI and pulmonary metastases on chest CT. Results: 48patients were included in the study (males

Page 44

= 14, females = 34; mean age 30 years, range 10-78 years), including 10 (20.5%) with LGCOS, 29 (60.5%) with POS and 9 (19%) with PeOS. The overall prevalence of skeletal metastases on BSc was 0% and pulmonary metastases on chest CT was 8%. 18 patients had WBMRI for skeletal staging, which did not demonstrate skeletal metastases.

Conclusion: Skeletal and pulmonary metastatic disease is extremely rare in non-conventional osteosarcoma, and therefore staging may not be required. However, larger studies are necessary to confirm this.

5. Multi-specialty surgical approach to patients with primary chest wall sarcoma

A. Bridgeman, Department of Thoracic Surgery, University Hospitals Bristol NHS Foundation Trust; S.Pradeep, Department of Plastic Surgery, North Bristol NHS Trust; A. Emam, Department of Plastic Surgery, North Bristol NHS Trust; T Wright(2), P Wilson(2), D West(1) 1 Department of Thoracic Surgery, University Hospitals Bristol NHS Foundation Trust 2 Department of Plastic Surgery, North Bristol NHS Trust Introduction Primary chest wall tumour resection is rare.

Specialist multi-disciplinary management is essential to ensure good outcomes and patient experience. The aim of this study was to review the surgical management of patients with primary chest wall tumours.

Methods A retrospective review of patients who underwent chest wall resection and reconstruction for primary chest wall tumour, from March 2012 to September 2018 was carried out. Hospital electronic records were scrutinized to collect data on histology, type of resection and reconstruction, length of stay, wound complications, and recurrence rates.

Results There were 31 patients who underwent chest wall resection and reconstruction in Bristol

at three different hospitals in our clinical network. Reconstruction was carried out using biosynthetic materials in 42% (n=13), combination of mesh and bone cement in 23% (n=7) and soft tissue cover in 23% (2 free flaps, 4 pedicled and 1 split-skin graft). 35% (n=11) of cases were joint operations. There was no perioperative mortality. Median length of hospital stay was 6 days (range 0-27). 16% (n=5) had wound complications necessitating re-operation. Final histology was 29% (n=9) soft-tissue sarcoma, 32% (n=10) bone sarcoma and 39% (n=12) benign. 13% (n=4) had R1 resection and 13% (n=4) developed recurrence within 2 years.

Conclusion A multidisciplinary surgical approach to patients with chest wall tumours is essential to ensure a low mortality and low early recurrence rate. Wound complications are reduced by combining thoracic surgery resection with plastic surgery reconstruction.

An individualized approach to collaborative perioperative strategy is feasible with good outcomes.

6. Management of epithelioid sarcoma

Andrew Knight, Leeds Teaching Hospital NHS Trust; Sean Botham, Leeds Teaching Hospital NHS Trust; Rachel Clancy, Leeds Teaching Hospital NHS Trust; James Smith, Leeds Teaching Hospital NHS Trust; Ian Smith, Leeds Teaching Hospital NHS Trust Introduction Epithelioid Sarcoma (ES), first described

by Enzinger in 1979 is rare tumour with an indolent course and a propensity to recur, it is often misdiagnosed and poorly managed. We describe the experience of one UK unit with presentation, management and outcomes of patients with ES as well as pertinent lessons learned.

Methods We carried out a retrospective review of patients presenting between 2010 to 2018, with a histological diagnosis of ES who were referred to our unit.

Page 45

Abstracts - Posters

Results: We identified 30 cases of anterior abdominal wall tumours (60% Fibromatosis, 40% Soft Tissue Sarcoma) managed in a single institution. Mesh reconstruction was used in 30% of cases. 0% wound complication rate has been reported. 0.03% recurrence rate has been reported.

Conclusion Epithelioid sarcoma has a “deceptively harmless appearance” and is often mistaken for a benign or inflammatory condition.

The reduction in funding from CCGs for the excision of so-called benign looking lumps and bumps may lead to a rise in late presentation, and hence timely treatment, of patients who turn out to have an ES. This is a cautionary tale and clinicians should have ES on their list of differential diagnoses, especially the young with a prolonged history of what appears to be an innocuous lump.

7. Quality Improvement in Sarcoma Diagnostic Pathway

Julie Woodford, London Sarcoma Service; Gemma French, London Sarcoma Service; Andy Johnston, London Sarcoma Service The London Sarcoma Service seized an opportunity to participate in a Quality Improvement Programme utilising a systematic approach to our 2 Week Wait diagnostic pathway. Our aims are to improve patient experience and reduce delays in diagnosis.

These goals are particularly relevant to specialist sarcoma services where there has been an overwhelming increase in new patient referrals. There are approximately 2000 new referrals to RNOH via the 2ww pathway who will require rapid access to assessment and

diagnostic tests. Despite the increased referrals there remains a low conversion rate to a Sarcoma diagnosis (5%). This is 1/5 of the total number of sarcoma diagnosed in the service.

Using a QI model a senior multi-professional project group aimed to develop an efficient and patient centred diagnostic pathway for patients with bone and soft tissue sarcomas.

We have reviewed and audited the current patient pathway, engaged with patients, and proposed service redesign to shorten the pathway from referral to diagnosis, thereby improving patient experience, reducing hospital visits and treating patients sooner.

Working with key stakeholders PDSA cycles were utilised to test the changes. These include triaging new referrals, developing a one stop diagnostic nurse led clinic and improving our patient tracking to expedite the sarcoma pathway or discharge the patient. An education programme is planned for primary care providers to reduce the number of inappropriate referrals to our service.

8. A review of the use of Doxorubicin and Olaratumab for advanced soft-tissue sarcoma in the North East

Dr Anna Stansfeld, Northern Centre for Cancer Care, Freeman Hospital, Newcastle; Dr Beth Lambourne, Northern Centre for Cancer Care, Freeman Hospital, Newcastle; Dr Talal Mansy, James Cook University Hospital, Middlesbrough; Dr Radha Todd, Northern Centre for Cancer Care, Freeman Hospital, Newcastle Introduction: Doxorubicin and olaratumab are being used in combination in the treatment of metastatic soft tissue sarcoma (STS). A 2016 Phase 1B/2 trial, showed a significant improvement in overall survival (11.8 months) with combination treatment compared to doxorubicin alone, with an objective response rate of 18.2%.

NICE (National Institute for Health and Care Excellence) approved this combination for first line treatment for metastatic STS in 2017 and it has been used in Newcastle and

Page 46

Middlesbrough since. We wished to review the outcomes for our patients compared to the trial data.

disease over a 7 year period. The therapy team suspected numbers are rising & wanted to review caseload and management pathways.

Methods and Results: Patients who had received doxorubicin and olaratumab were identified retrospectively using Chemocare software. 26 patients (18F, 8M), aged between 25 and 82 years old, were identified. 5 patients (19%) discontinued treatment prior to response assessment due to poor performance status and clinical progression. 1 patient is awaiting a response assessment. Of the 20 patients who had CT response assessments after Cycle 3 or 4, the results were as follows: CR 0%, PR 30%, SD 35%, PD 35%. Objective response rate was 30%. 5 patients (19%) are continuing on doxorubicin/olaratumab. 4 (15%) are on next line chemotherapy. 14 (54%) of patients have died – 4 whilst on treatment, and 10 who had been discharged for best supportive care (BSC).

3 patients (12%) are receiving ongoing BSC.

Methods: Retrospective data collection January to July 2018. Patients identified from archived operation lists. Data collected: gender, age, diagnosis, presence of pathological fracture, length of stay pre and post operatively , use of hip abduction brace, dislocation, survival, functional status upon discharge , discharge destination, onward referral to physiotherapy.

Conclusion: Objective response rate in the Phase 2 study was 18.2%. Our data demonstrates similar positive responses and toxicity profile. This therapeutic combination requires further review as the patient cohort increases in numbers.

Results: 38 proximal femoral replacements 28 metastatic cancer, 10 sarcoma 22 cases admitted after sustaining pathological fracture.

RNOH hospital standard LOS for PFR 10 days Metastatic average LOS post op 17. days, sarcoma average LOS 13.5 days 19 cases provided with hip abduction brace post op 25 patients discharged home 25 people alive at 6 months Conclusion: Significant increase in volume of PFRs for metastatic disease

In 2009 8 metastatic patients underwent PFR In 2018 28 metastatic patients underwent PFR Percentage yearly increase of 115% Chances of local services managing these people for rehabilitation increasing Complexity of metastatic patients potentially different to primary

9. Service Evaluation of incidence and management of proximal femoral replacements (PFR) for metastatic bone disease versus sarcoma

Aoife O’Meara, Royal National Orthopaedic Hospital

10. Dermatofibrosarcoma Protuberans (DFSP) in Children: A combined multidisciplinary approach to rare tumors

Aim: Assess number of PFR’s completed for metastatic bone disease versus sarcoma over 1 year.

Background: Improvements in cancer therapies & life expectancy is resulting in more people living with metastatic bone disease. In 2009 Ashford et al published a paper from RNOH outlining 62 patients underwent PFR for metastatic

Aseel Sleiwah, Guy’s and St Thomas’ NHS Foundation Trust; Emma Craythorne, Guy’s and St Thomas’ NHS Foundation Trust; Catherine M. Stefanato, Guy’s and St Thomas’ NHS Foundation Trust; William Rickaby, Alistair Robson, Aina Greig Guy’s and St Thomas’ NHS Foundation Trust Introduction:

Page 47

Abstracts - Posters

Results Patient age range was between 12-24 years with an average time from initial presentation to diagnosis of 26 months. Initial diagnoses were unspecific lump, pyogenic granuloma and recurrent wound infection. Patients were seen by their general practitioner, dermatology, and orthopaedics before referral and definitive treatment by plastic surgery. Despite wide excision, margins were positive in 50% of patients. Even those with clear margins and complex reconstructions had high rates of recurrence (30%). Outcomes were poor, with local and regional recurrence, metastatic spread and death (15%).

Aims: The aim of this study is to share our experience in management of complex cases of DFSP in a combined surgical approach involving a plastic and a dermatological surgeon, using Slow Mohs Micrographic Surgery (MMS).

Methods: A retrospective review of the notes of three children with DFSP was conducted. The tumor was excised by the operating team using slow MMS technique. This method integrates surgical experience, familiarity with various anatomical sites, excision technique and high volume of cases between the two disciplines. Results All three cases underwent excision of the tumor under general anesthetic using Slow MMS as a standard technique. The specimens were mapped and processed for histopathological examination. The number of excisional stages ranged between (1-2). The resultant defect was reconstructed with a dermal regeneration matrix (Integra and Matriderm) and a split skin graft. Specimens were process using CD34 immunohistochemistry and FISH COL1A1 gene translocation studies.

Conclusion: A combined multidisciplinary approach involving plastic and dermatological surgeons sets a new standard to treat complex rare cases of DFSP in children to achieve smaller defect with good margin control.

11. Rates of Osteointegration of Hydroxyapatite-Coated Collars in Cemented Massive Endoprostheses Following Revision Surgery

Benjamin Davies, Royal National Orthopaedic Hospital; Andrew Johnston, Royal National Orthopaedic Hospital; Christian Gray

Stephens, Royal National Orthopaedic Hospital; Loukas Andritsos, Royal National Orthopaedic Hospital; Rajiv Kaila, Royal National Orthopaedic Hospital; Gordon Blunn, University of Portsmouth School of Pharmacy and Biomedical Sciences; Craig Gerrand, Royal National Orthopaedic Hospital; Panos Gikas, Royal National Orthopaedic Hospital Introduction: Hydroxyapatite-coated collars on massive endoprostheses are designed to reduce aseptic loosening by enhancing bone on-growth at the bone-implant shoulder, reducing incidence of radiolucent lines adjacent to the boneprosthesis and stem-cement interface (Coathup et al., 2015). There is a paucity of research regarding benefit in revision surgery. We aimed to assess bone on-growth at hydroxyapatitecoated collars of cemented massive endoprostheses following revision surgery.

Methods: A retrospective review of all massive endoprosthesis implantation for revision purposes at our centre between 2010-2017 was undertaken. Collar-bone interface was analysed using most recent plain radiographs of cases with at least 6 months radiological follow-up. Osteintegration was classified by number of osteointegrated collar sides (0-4) and using Sankar et al(2012) grading.

Results and discussion: 41 cases with mean follow-up 44 months (range 8-94) were reviewed. 25 were distal femoral replacements, 10 proximal femoral replacements, 4 proximal tibial replacements 1 tibial diaphyseal replacement and 1 humeral diaphyseal replacement. 58.5% showed no osteointegration on any collar side, of which 70.8% had bone growth with radiolucency between bone growth and collar. Sanker et al. (2012) reported 100% revision rate over 5 years in this subgroup. Patients undergoing revision for aseptic loosening showed lower, but not significant, rates of osteointegration (25%) (infection (58.3%), periprosthetic fracture (60.0%), implant failure (42.9%)).

Conclusion: This study highlights poor osteointegration

Page 48

to hydroxyapatite-coated collars in massive endoprostheses for multiply operated cases, independent of indication. Changes to collar design and further clinical correlation with clinical associations may improve osteointegration in revision surgery.

12. The role of Evicel® (fibrin sealant) in bone and soft tissue tumour surgery

Benjamin Davies, Royal National Orthopaedic Hospital; Amir Ardakani, Royal National Orthopaedic Hospital; Christian Gray Stephens, Royal National Orthopaedic Hospital; Jakub Jagiello, Royal Adelaide Hospital; William Aston, Royal National Orthopaedic Hospital; Panos Gikas, Royal National Orthopaedic Hospital; Tim Briggs, Royal National Orthopaedic Hospital; Rob Pollock, Royal National Orthopaedic Hospital Introduction: Bone and soft tissue tumour surgery carries high risk of intra-operative blood loss, and therefore anaemia, transfusion and wound complications. Evicel®, a topical haemostatic agent, used intra-operatively may help prevent bleeding and wound complications. We aimed to assess the use of Evicel® for haemostasis in orthopaedic tumour surgery.

Methods: A retrospective review of all patients undergoing resection of bone or soft tissue tumours receiving Evicel® in our centre between 2013-16 were identified and matched to patients undergoing similar procedures without Evicel®. Clinical factors analysed: Hb drop, drain output and length of use, transfusion requirement and wound complications.

developed wound complications, compared with 0/5 with. 0 patients with Evicel® required post-operative transfusion, compared to 2 without. Massive endoprosthesis implantation – Evicel® reduced post-operative transfusion requirements (48.2% to 18.5%, p=0.0005) and wound complications (15% to 0%, p=0.0013).

Large soft tissue tumour resection (>300cm3) - post-op transfusion requirements (30% to 4.7%, p=0.002) and drain output reduced (p=0.28) with Evicel® use. Large bone tumour resection (>200cm3) – Evicel® showed reduced transfusion requirement (67% to 44%, p=ns).

Conclusions: There are possible benefits for using Evicel® in procedures associated with large soft tissue flaps. Further studies should consider the use of Evicel® in these patient groups.

13. Trabectedin: Based on 8 years of experience, is it worth offering this chemotherapy to patients without L-type soft tissue sarcomas?

Beth Lambourne, Freeman Hospital NHS; Sally Hall, Freeman Hospital NHS; Lambourne B 1, Hall S 1, Mansy T 2, Margetts J 1, Todd R 1, Verrill M 1. 1 Northern Centre for Cancer Care, Freeman Hospital, Newcastle, UK 2 James Cook Hospital, Middlesbrough, UK Background Trabectedin is an alkylating agent and is NICE approved as 2nd line treatment for inoperable soft tissue sarcomas (STS). STS are a heterogenous group with variable response rates to 1st and 2nd line chemotherapy. A retrospective review has been undertaken in 2 cancer centres in the North East to assess its efficacy over an 8 year period.

Results and discussions: 240 cases using Evicel® were matched to 189 without. Overall, there was a significant reduction in post-operative transfusion (12% vs 29%, p=0.0001), but not in post-operative wound complications (15% vs 20%) in patients receiving Evicel®. Differences were noted following specific surgeries: Hindquarter amputation - 4/4 cases without Evicel®

Methods All STS patients treated with Trabectedin between 2010 and 2018 were included.

Data collected included demographics, histopathology, number of cycles received, recorded best response on imaging (not RECIST) and survival (from date of 1st Trabectedin dose to death).

Page 49

Abstracts - Posters

Dermatofibrosarcoma protuberance (DFSP) is a rare subtype of sarcomas compromising (1-6%) of all sarcomas. It is a locally aggressive tumour with a low risk of metastasis. It mainly affects adults between 20-40 years of life.

The incidence in children is less than one per million before age 20 years.

Conclusion In keeping with published data, Trabectedin shows increased activity in L-Type sarcomas (excluding uterine leiomyosarcoma) with encouraging activity in synovial sarcomas also. Due to the lack of a viable alternative, we continue to offer this chemotherapy to patients with all histological subtypes of STS. 14. Role of Bisphosphonates as Adjuvants of Surgery in Giant Cell Tumor of Spine

Chaitanya Dev Pannu, Royal National Orthopaedic Hospital, London; Pankaj Kandhwal, All India Institute of Medical Sciences, Rishikesh; Vijay Raghavan, All India Institute of Medical Sciences, New Delhi; Shah Alam Khan, All India Institute of Medical Sciences, New Delhi, Shishir Rastogi, All India Institute of Medical Sciences, New Delhi, Arvind Jayaswal, All India Institute of Medical Sciences, New Delhi Background: The role of bisphosphonates is well established in giant cell tumor of bone (GCTB) of extremities, but its role in spine GCTB is still not established. Our main purpose was to evaluate the role of bisphosphonates in spinal GCTB with the help of radiologic assessment.

Methods:

A retrospective analysis of all spine GCTB patients who underwent an operation from July 2005 to July 2014 was done. Patients of spine GCTB in whom bisphosphonates were given constituted the study group. This group was compared to patients in whom bisphosphonates were not given. Preoperative and postoperative radiographs and CT scans were studied. A thorough evaluation of the presence of sclerosis was done on them. Bisphosphonates were considered to be effective if either sclerosis or new bone formation was present.

Results: A total of 13 cases of spine GCT underwent operation from July 2005 to January 2014. All patients of GCTB spine who underwent an operation after 2008 at our institute were given bisphosphonates postoperatively. Of 13 cases, bisphosphonates were given postoperatively in 6 patients: 5 patients were female and 1 patient was male. Of these 6 patients, 3 patients had sacrum GCTB and 1 patient each had T9, T11, and L5 vertebrae GCTB. The average follow-up period was 39.33 months (minimum follow-up was 18 months and the maximum follow-up was 72 months). Postoperative sclerosis was present in all 6 patients. No recurrence of the tumor was present in the bisphosphonate group, but 2 patients had a recurrence in the group that did not receive bisphosphonates.

Conclusions: Bisphosphonates are effective and safe adjuvant therapy along with appropriate surgical intervention in spinal GCTBs and may have a role in decreasing the recurrence of this tumor.

15. Sarcoma: A Head To Toe Approach

Maniram Ragbir, Newcastle Upon Tyne NHS Foundation Trust; Christina Whitehead, Newcastle Upon Tyne NHS Foundation Trust; Iain Anderson, Newcastle Upon Tyne NHS Foundation Trust; Jackie Callear, Newcastle Upon Tyne Hospitals NHS Foundation Trust; Timothy Crowley, Newcastle Upon Tyne Hospitals NHS Foundation Trust; Ashish Magdum, Newcastle Upon Tyne Hospitals NHS

Page 50

Foundation Trust Introduction Sarcomas account for less than 1% of malignant neoplasms in the UK. As the primary aim of surgical intervention is clear oncological margin, tumourresection often leaves large defects, creating a reconstructive challenge.

We present our experience of 98 cases of free flap reconstruction following sarcoma excision in the North of England Bone and Soft Tissue Tumour Service.

Method A retrospective study of free flap reconstruction in sarcoma surgery was conducted between 2005 and 2018. 98 cases - 56 males and 38 females, with a mean age of 57 years - underwent sarcoma excision and free tissue reconstruction to various anatomical locations. Medical notes and our local database were examined for details of sarcoma type, resulting defect following excision, choice of free flap, complications, disease-free survival and overall survival.

Results 13 different free flaps were used for reconstruction. The most common choices were antero-lateral thigh (n=40), latissimus dorsi (n=24), fibula osteo-cutaneous (n=10) and rectus abdominus muscle flaps (n=10). 10 (10.2%) returned to theatre with an overall flap loss rate of 4%. Mean disease free survival rate was 980 days.

Conclusions Our data shows that microvascular techniques can be employed in a wide variety of challenging reconstructive situations following sarcoma resection with an acceptable complication profile and outcomes. This broadens the options for limb salvage or functional reconstruction. We discuss when free tissue transfers are appropriate and our experience of free flap choice.

16. Cardiac intimal sarcoma- a case report

Christine Gray, University Hospitals Birmingham, NHS Foundation Trust; David

Peake, University Hospitals Birmingham, NHS Foundation Trust; Jenny Sherriff, University Hospitals Birmingham, NHS Foundation Trust; Dr Mariam Jafri, University Hospitals Birmingham, NHS Foundation Trust Primary cardiac tumours are rare. The majority are benign, but of the 25% which are malignant, sarcomas are the most prevalent.

Intimal sarcomas tend to occur in large arteries; however, there are case reports of cardiac intimal sarcomas which are typically associated with poor prognosis. Surgery remains the mainstay of treatment; however, there are no clear guidelines on the role of adjuvant chemotherapy or radiotherapy.

In this report we describe the case of a 31 year old lady who presented with a transient ischaemic attack. Her echocardiogram demonstrated a 3.3 cm x 4.8 cm left atrial mass which was initially thought to be a myxoma.

She underwent open cardiac surgery when the lesion was removed alongside the involved wall of the left atrium. Histology, confirmed the diagnosis of a grade 3, intimal cardiac sarcoma with uncertainty regarding resection margins.

After multidisciplinary discussion across different NHS health trusts, it was decided to proceed with adjuvant chemoradiotherapy.

The patient received six cycles of ifosfamide, and radiotherapy was added with cycle two.

She received 60Gy in 30 fractions, which was delivered as intensity modulated radiation therapy. It is now 13 months since completion of her adjuvant treatment, and surveillance ECHO, cardiac MRI and CT, show no evidence of local or distant disease relapse. This case aims to describe the presentation of a rare, aggressive tumour and to discuss the options for management in a young patient with good performance status.

17. The effect of crizotinib on an ALKrearranged inflammatory myofibroblastic tumour- a case report

Christine Gray, University Hospitals Birmingham, NHS Foundation Trust; David Peake, University Hospitals Birmingham, NHS Foundation Trust; Jenny Sherriff, University Hospitals Birmingham, NHS Foundation Trust Page 51

Abstracts - Posters

Results Data was collected for all 76 patients treated with Trabectedin (29 male, 47 female). Median age 58 (range 22-84). Histology breakdownleiomyosarcoma 29%, uterine leiomyosarcoma 13%, liposarcoma 22%, synovial 16% and others 20%. There was significant variability in response rates and survival. This was best demonstrated when comparing liposarcomas and uterine leiomyosarcomas. The average number of cycles received being 8 in patients with liposarcoma (range 1-51) versus 3.6 (1-21) in the patients with uterine leiomyosarcoma.

This was also replicated with average survival being 474 days (range 11-1550 days) in the patients with liposarcoma versus 152 days (range 9-705 days) in the patients with uterine leiomyosarcoma.

in soft-tissue sarcoma research, where the projected demographic shift in population age distributions will be accompanied by increasing levels of comorbidity. This pilot study aims to summarise the co-morbid state of patients undergoing definitive surgical resection of a soft-tissue diagnosis and to investigate the relationship between comorbidity and all-cause mortality.

18. Comorbidity in Soft Tissue Sarcoma: A Pilot Study

19. Pre-operative biopsy practice and accuracy in a Retroperitoneal Sarcoma (RPS) unit

Ciaran Grafton-Clarke, University of Leicester; Lucie Wright, East Midlands Sarcoma Service; Anna Raurell Rosique, East Midlands Sarcoma Service; Kathryn H Steele, East Midlands Sarcoma Service; Thomas A McCulloch, East Midlands Sarcoma Service; Robert U Ashford, East Midlands Sarcoma Service

38 patients receiving a primary diagnosis of a soft-tissue sarcoma in 2017 were included in the primary analysis, of which the most common primary diagnoses were atypical lipomatous tumour (n = 9) and leiomyosarcoma (n = 6). The average age of participants was 55.9 years (SD 20.3), average BMI 28.1 kg/m2 (SD 4.0), smoker (> 5 cigarettes per day) 18.2%, high alcohol intake (> recommended unit intake) 50%, average Charlson Comorbidity Index score 4.1 (SD 2.1) and average ASA physical status score 1.1 (SD 0.8). Since surgical resection, 2 patients have passed away, of which both had a high CCI and ASA status (CCI: 5, 6; ASA: 3, 3). This pilot study, while performed on a small scale, summarises the co-morbid state of all patients undergoing definitive resection in 2017 covering six local trusts.

This pilot represents a primer to a larger study, whereby disease-specific and overall-mortality rates in relation to an array of co-morbidities will be investigated, with the aim of identifying prognostic indicators for mortality and complication rates.

Eleanor Pettitt, University of Nottingham; Alastair Simpson, Nottingham University Hospitals NHS Trust; David Humes, University of Nottingham; Catherine Walter Nottingham University Hospitals NHS Trust

Comorbidity is an important prognostic factor for survival in numerous cancer types. This relationship is heavily under-investigated

Background: NHS England’s Sarcoma Service Specification recognises that all retroperitoneal sarcomas (RPS), when not radiologically

Page 52

liposarcoma (LPS), should be biopsied prior to excision to determine histology. Previous studies have suggested that pre-operative biopsies can misdiagnose dedifferentiated (DD) LPS as well-differentiated (WD) LPS in around half of all patients.1 This study aimed to assess the use and accuracy of pre-operative biopsy in a UK centre.

Methods: All patients with a new diagnosis of RPS between 2008-2017 were identified from the East Midlands Sarcoma Service database.

Retrospective data on patients’ pre-operative radiological diagnosis, biopsy status, histology and final surgical histology were recorded from hospital records. Basic analysis was carried out using excel.

Results: Of 60 patients who underwent resections of primary tumours, 37 (61.7%) had a preoperative biopsy. Of the 23 patients who did not undergo pre-operative biopsy, 20 were radiologically LPS therefore did not require a pre-operative biopsy, the remaining 3 (2 leiomyosarcomas, 1 fibrosarcoma) were not amenable to image-guided biopsy. In 7 biopsies (18.9%) there was mismatch between histologic subtype at biopsy and final surgical histology. Of the 5 patients whose preoperative biopsies demonstrated WD LPS, none went on to have dedifferentiation at final postoperative histology.

Conclusion: This unit’s biopsy practice follows RPS service specification guidance and demonstrates 80% of all pre-operative biopsies correctly identify the final post-operative histology. Although low-numbered, this centres data differed from other studies and did not demonstrate DDLPS being misdiagnosed as WDLPS at biopsy. Reference 1 Diagnositic accuracy of percutaneous biopsy in retroperitoneal sarcoma. Tattersall H, Almond M et al. BSG Annual Conference 2018 Programme and Abstract Book. 2018: 37 20. Survival Outcomes after Retroperitoneal Sarcoma (RPS) surgery in a single UK centre

Eleanor Pettitt, University of Nottingham; Alastair Simpson, Nottingham University Hospitals NHS Trust; David Humes, University of Nottingham; Catherine Walter, Nottingham University Hospitals NHS Trust Background: Reports of 30-day mortality and overall survival rates following potential-curative retroperitoneal sarcoma (RPS) resection vary in the literature. In 2016 the “Multi-institutional Collaborative RPS Working Group” reported 1.8% 30-day post-operative mortality and 67% 5-year overall survival.1 The aim of this study was to investigate 30-day post-operative mortality and overall survival rates following RPS resections within a single UK centre.

Methods: All primary malignant RPS undergoing surgery between 2008-2017 in a single UK centre were identified from the unit’s prospectively collected database. Additional retrospective data on follow-up outcomes was collected from hospital electronic systems. Nonparametric survival analyses were carried out.

Results: Of 60 primary RPS resections undertaken during this period, 16 were well-differentiated liposarcoma, 21 were de-differentiated liposarcoma, 13 were leiomyosarcoma and 10 were other histologic subtypes. Median followup from surgery was 25 months (interquartile range (IQR) 11-49). Thirty-day post-operative survival rate was 100%. One, 2 and 3-year overall survival (OS) were 88% (95% CI, 76, 94), 74% (95% CI, 59, 84) and 66% (95% CI, 51, 78) respectively.

Conclusion: These data show two-thirds of patients undergoing potentially-curative retroperitoneal sarcoma resections at this unit were alive at three years post-surgery. These data are similar to other published data.1 Reference 1. Gronchi A, Strauss DC, Miceli R, Bonvalot S, Swallow CJ, Hohenberger P, et al. Variability in Patterns of Recurrence After Resection of Primary Retroperitoneal Sarcoma

Page 53

Abstracts - Posters

Inflammatory myofibroblastic tumours (IMTs) are a type of mesenchymal neoplasm consisting of proliferating spindle cells within an inflammatory environment. They typically occur within the abdominopelvic region, retroperitoneum and lung. Fifty percent of IMTs demonstrate a rearrangement within the ALK gene on chromosome 2p23 and there is recent evidence for the use crizotinib as the standard of care in this group of patients who are felt to be inoperable. In this report we describe the case of a 37 year old lady with a radiologically complete response to crizotinib. She initially presented in 2016 with mild hypertension and abdominal ultrasound revealed a large abdominal mass. CT further assessed the lesion and demonstrated complex mesenteric masses within the root of the small bowel mesentery.

Biopsy confirmed the diagnosis of an IMT with ALK rearrangement; however, her tumour was considered inoperable due to proximity of the superior mesenteric vessels. In January 2017, she commenced oral crizotinib at a dose of 250mg twice daily. An early CT-PET scan, three weeks after commencing treatment, showed an excellent partial response in both the size of the lesion and the FDG uptake (SUV dropped from 22.7 to 3.9). Subsequent CT scans have continued to show ongoing improvement and a CT scan in September 2018, 20 months following commencement of crizotinib, reported no measurable disease. This case aims to describe a case of an inoperable IMT with ALK rearrangement which demonstrates an excellent, sustained response to crizotinib treatment.

21. Complications following excision of bone and soft tissue sarcoma of the extremities and incidence of postoperative Lymphedema

Fabio Nicoli, Newcastle upon Tyne Hospitals NHS Trust,UK; Kenneth Rankin, Newcastle upon Tyne Hospitals NHS Trust,UK; Craig Gerrand, Newcastle upon Tyne Hospitals NHS Trust,UK; Maniram Ragbir, Newcastle upon Tyne Hospitals NHS Trust,UK Introduction Bone and Soft tissue sarcomas comprise of a group of rare malignant tumours, which occur most commonly in the extremities. Limb preservation surgery can be combined with an adjuvant preoperative or postoperative radiation therapy. However, this treatment carries significant risks of functional disability and further complications. Lymphedema can be a troublesome and important complication of limb salvage treatment after bone and soft tissue sarcoma. Previous sarcoma studies reported an incidence of lymphoedema between 19% and 30%.

The objective of this study is to identify complications of patients with bone and soft tissue sarcomas in the extremities.

The incidence and severity of patients with lymphedema post-surgery was evaluated, including the associated risk factors such as chemotherapy, radiotherapy and extent of excision.

Methods A retrospective analysis of the tumour database was utilized to identify patients who had undergone surgical excision of sarcoma of the bone or soft tissue of the extremities. The survey was conducted at the North of England bone and soft tissue tumour service, between July 2012 and July 2017.

Results A total of 310 patients met the inclusion criteria. Surgical procedures consisted of 191 (61%) wider excision, 92 (30%) excision, 24 (8%) amputation, 3 curettage (1%). The survival rate

at 5 years was 74%. However, the remaining 26 % met a median life expectancy of 15 months post-surgery. 124 patients (40%) experienced early complications such as infections, wound dehiscence, seroma, hematoma, bleeding amongst others. Out of 286 patients, 50 (17,5%) developed lymphedema as a late complication.

Conclusion The results showed a high rate of multiple early complications with lymphedema representing the most common late complication. Therefore, the study highlights the need further research into the education, management and treatment of lymphoedema, in order to provide evidencebased practice, extend patient awareness and their overall quality of life.

22. Treatments for Low Grade Fibromyxoid Sarcoma: Systematic Review And Experience of a Tertiary Cancer Centre

Florence Chamberlain, The Royal Marsden Hospital NHS Foundation Trust; Bodil Engelmann, The Royal Marsden Hospital NHS Foundation Trust; Spyridon Gennatas, The Royal Marsden Hospital NHS Foundation Trust; Omar Al-Muderis, The Royal Marsden Hospital NHS Foundation Trust; Charlotte Benson, The Royal Marsden Hospital NHS Foundation Trust; Robin L Jones, The Royal Marsden Hospital NHS Foundation Trust Introduction: Low-grade fibromyxoid sarcoma (LGFMS) is an indolent and rare soft tissue sarcoma subtype. Complete surgical resection with or without radiation is the advised management for localized disease. Treatment options for advanced or metastatic disease are limited.

The aim of this study was to document the outcome of patients with LGFMS and the efficacy and safety of non-surgical treatments in advanced disease.

Methods: A literature search identified articles using MESH terms low-grade fibromyxoid sarcoma, LGFMS, radiotherapy and chemotherapy.

We performed a retrospective review of a

Page 54

prospectively maintained database which identified 102 LGFMS patients treated at our institution from 1994-2018.

Results: Median time from presentation to death or last follow up was 45.3 months (interquartile range (IQR) 12.1-82.3 months). 93 (91%) had primary resection of which 17.2% (16/93) required re-excision. Median time from presentation to death or last follow up was 49.9 months (IQR 13.5 – 88.8 months) following excision and 29.4 months (IQR 7.5 – 42.3 months) for those who did not undergo excision. Seven (6.8%) patients had palliative systemic therapy and 21.6% (22/102) had radiotherapy. Median time from presentation to death or last follow up in patients treated with chemotherapy and radiotherapy was 45.8 months (IQR 30.3 – 95.8 months) and 45.8 months (IQR 21 – 129.7 months) respectively.

Conclusions: Our descriptive data support the current opinion that chemotherapy, hormone treatments and radiotherapy are inactive in advanced LGFMS. Surgical resection remains an effective treatment for localized LGFMS. Further studies are required to determine if targeted treatments can play a role in management.

23. Use of Regorafenib in Patients with Gastro-Intestinal Stromal Tumour (GIST): Experience Of Toxicity Management in a Tertiary Cancer Centre

Constance Williams-Sharkey, The Royal Marsden Hospital; Cecilia Collingwood, The Royal Marsden Hospital; Lucia Chen, The Royal Marsden Hospital; Florence Chamberlain, The Royal Marsden Hospital; Bodil Engelmann, The Royal Marsden; Hospital Sonia Mansukhani, The Royal Marsden Hospital; Omar Al-Muderis, The Royal Marsden Hospital; Alison Dunlop, The Royal Marsden Hospital; Dharmisha Chauhan, The Royal Marsden Hospital; Spyridon Gennatas, The Royal Marsden Hospital; Robin L Jones, The Royal Marsden Hospital; Charlotte Benson, The Royal Marsden Hospital

Introduction: Regorafenib is a multi-kinase inhibitor approved as 3rd line treatment for metastatic GIST. Dose limiting toxicities are frequently seen and many patients require dose reductions. The study aimed to evaluate the toxicity of regorafenib in a real world GIST population.

Method: Retrospective review of a prospectively maintained database identified 50 patients with GIST treated with regorafenib at our institution between March 2013 and September 2018.

Results: Baseline median ECOG performance status was 1. Median time from presentation to death or last follow up was 87 months (IQR 55-127 months). Median duration of regorafenib treatment was 6.7 months (IQR 1.9-12.2 months). The main reason for discontinuing regorafenib was progressive disease (PD) (38/50 (76%)). Other reasons were toxicity (11/50 (22%)), death (3/50 (6%)) and withdrawal of consent (1/50 (2%)). Grade 3-4 AEs were seen in 22/50 (44%) patients; palmarplantar erythrodysesthesia was most frequently seen (10/50 (20%)). Fatigue (6/50 (12%)), hypertension (5/50 (10%)), hepatotoxicity (2/50 (4%)) and diarrhoea (1/50 (2%)) were also seen. Dose reductions were required in 20/50 (40%) patients and 8/50 (16%) patients started regorafenib at a lower dose band than the recommended dose (160mg) due to comorbidities or concern over a higher individual risk of toxicity (2 patients started on 120mg, 6 patients started on 80mg).

Conclusion: Although PD was the main reason for discontinuing treatment in this study, toxicity management and dosing of regorafenib remain critical. Median duration of treatment was longer compared to the GRID trial population (28.9 vs 22.9 weeks) suggesting durable clinical benefit with regorafenib 24. Scalp angiosarcoma: challenges of bolus application and comparison of two bolus techniques

Page 55

Abstracts - Posters

(RPS). Ann Surg. 2016;263(5): 1002-1007

Treatment of scalp angiosarcoma should include surgery to achieve wide local excision, however margins may be compromised because of the infiltrative nature of the disease and anatomical constraints. Post-operative radiotherapy (RT) is often employed to reduce the risk of local recurrence which can be as high as 62%.1 The challenges of RT include achieving homogenous dose coverage given the convexity of the scalp and conformal bolus application to increase the skin dose.

The standard technique was mixed photon and electron fields, however IMRT/ VMAT have been shown to give better conformity and more homogenous dose distribution.2 Application of conventional bolus material such as wax or superflab can be effective but achieving close apposition to the skin and reproducible set-up is difficult and frequently leaves air gaps which impair dose coverage. Hadziahmetovic et al. proposed the scalp uniform bolus application (SCUBA) technique of using neoprene dive hoods rather than conventional wax bolus.3 We present a dosimetric and clinical comparison of the two bolus techniques in a patient with extensive scalp angiosarcoma. A 64 year old man had experienced rapid relapse of scalp angiosarcoma following an extensive resection and flap reconstruction and re-excision had left positive margins. He was treated with whole scalp radiation to a dose of 60Gy in 30 fractions with a 5Gy integrated boost to the margin-positive areas. Comparison of VMAT plans using both wax and neoprene bolus confirmed that neoprene provides a conformal bolus application with the elimination of air gaps and was well tolerated by the patient.

1. Patel S, Hayden R, Hinni M et al.

Angiosarcoma of the Scalp and Face: The Mayo Clinic Experience. JAMA Otolaryngol Head Neck Surg. 2015;141(4):335-340 2. Wojcicka J, Lasher D, McAfee S and Fortier G. Dosimetric comparison of three different treatment techniques in extensive scalp lesion irradiation. Radiother Oncol. 2009

May;91(2):255-60 3. Hadziahmetovic M, Weldon M, Pearson M, Werner P and Siddiqui F. Scalp uniform bolus application (SCUBA) technique for homogenous scalp and regional nodal irradiation. Practical Radiation Oncology (2014) 4, e95–e99 25. Introduction of Treatment Summary at RJAH

Geraint Davies, Robert Jones & Agnes Hunt Orthopaedic Hospital; Miss Karen Shepherd, Robert Jones & Agnes Hunt Orthopaedic Hospital At the Robert Jones & Agnes Hunt Orthopaedic Hospital, we have introduced a detailed treatment summary to improve the communication between our services, GP’s and the patient. The document consolidates all the information we have previously given to patients. The document forms part of the recovery package initiative and is to inform GP’s and other professionals of any actions they need to take following episode of care at our Hospital and who specifically to contact with questions and concerns. The patient will also receive a copy of the summary to improve understanding of their surgery and condition and information regarding any ongoing referrals to other services. The document includes summaries of all services involved in their care during an episode and any recommendations for the future.

26. Lorlatinib for the treatment of metastatic inflammatory myofibroblastic tumour: a case report

Han Hsi Wong, Cambridge University Hospitals NHS Foundation Trust; Ramesh Bulusu, Cambridge University Hospitals NHS Foundation Trust; Gail Horan, Cambridge University Hospitals NHS Foundation Trust; Gloria Anyaegbu, Dochka Davidson, Agnes Bautista, Charlotte Harvey-Wright, Sarah Prewett, Jennifer Harrington, Helen Hatcher Cambridge University Hospitals NHS Foundation Trust Inflammatory myofibroblastic tumour (IMT)

Page 56

is a rare malignancy with limited responses to corticosteroids and chemotherapy. About 50% of cases have activating rearrangements in the ALK gene. Response to ALK inhibitors such as crizotinib has been reported but these are not available on the NHS. A 40-year-old man presented with cough and abnormal chest X-ray – CT showed a large right upper lobe mass with nodal, trapezius and cerebral metastases. He has a history of metastatic testicular embryonal carcinoma 8 years ago (treated with bleomycin, etoposide and cisplatin). Biopsy of the lung and trapezius masses confirmed IMT. Next-generation sequencing demonstrated TPM4-ALK gene fusion. He was treated with prednisolone without clinical benefit. He then received entrectinib, an experimental inhibitor of Trk, ROS1 and ALK in an early-phase trial but his disease progressed in less than 3 months.

Ifosfamide and etoposide in addition to radiotherapy to the brain and chest were administered. Transient improvement in the radiotherapy-treated area was observed but shortly afterwards his disease progressed on all sites including the development of a new adrenal metastasis. Compassionate use of the third-generation ALK inhibitor lorlatinib (100 mg od) resulted in disease improvement on all sites after 8 weeks, including a partial response of his brain metastasis, although he developed significant unilateral right-sided organising pneumonia that was thought to be related to infection and previous radiotherapy. At the time of writing, his disease has remained stable 6 months after he started lorlatinib. Toxicities were mild and included G1 oral stomatitis, insomnia, paronychia and G2 hyperlipidaemia.

27. Perivascular Epithelioid Cell Tumour (PEComa): Presentation, Treatment and Outcome in a Single Institution

Han Hsi Wong, Cambridge University Hospitals NHS Foundation Trust; Ishan Mehta, Cambridge University Hospitals NHS Foundation Trust; Dochka Davidson, Cambridge University Hospitals NHS Foundation Trust; Jennifer Harrington, Sarah Prewett, Gail Horan, Helen Hatcher, Cambridge University Hospitals NHS Foundation Trust

Background: PEComas are rare tumours driven by the tuberous sclerosis complex gene mutations.

They have variable clinical behaviour and responses to treatment.

Methods: Retrospective analysis of presentation, treatment and outcome of our patients over a 6-year period.

Results: Five patients were identified (3 females, 2 male). Median age at diagnosis was 59 years. A man with a 6 cm periscapular mass was treated with surgery and adjuvant radiotherapy, and remains disease-free 5 years after diagnosis. Another man had a 6 cm PEComa resected from his leg followed by adjuvant radiotherapy, with no recurrence at 2 years.

Two patients (one with tuberous sclerosis) had uterine PEComa, treated with hysterectomies.

One has stable lung nodules after 2 years follow-up but the other developed lung and abdominal metastases 2 months following hysterectomy. She did not respond to either sirolimus or pazopanib and died 21 months after diagnosis. A 65-year-old female had resection of liver PEComa (incidental finding during cholecystectomy), developing lung and liver metastases 4 years later. Sirolimus (2mg od) resulted in excellent response but she developed drug-related pneumonitis.

Treatment was stopped to allow pneumonitis resolution but was restarted again at 1 mg due to PEComa progression. Disease stabilisation was achieved although the pneumonitis recurred. Following interruption of sirolimus, her disease again progressed and she is currently being treated with concomitant prednisolone and sirolimus 0.5mg.

Conclusion: Complete surgical resection is the primary treatment of choice. Sirolimus has shown excellent activity in one patient but is hindered by drug-induced pneumonitis.

28. Primary Abdominal Wall Sarcomas: An experience of 57 patients surgically treated

Page 57

Abstracts - Posters

Gareth Ayre, Bristol Cancer Institute; Paula Wilson, Bristol Cancer Institute; Allan Day, Bristol Cancer Institute; Alison Stapleton, Bristol Cancer Institute

wall sarcomas. Larger tumours (?5cm) and high grade tumours are associated with a poorer prognosis in terms of both DFS and OS.

Hannah Tattersall, University of Birmingham; Samuel J. Ford, Midlands Abdominal and Retroperitoneal Sarcoma Unit; Alessandro Gronchi, Istituto dei Tumori, Milan;

29. Bayley Walker Proximal Humeral Reconstruction – The Stanmore Experience

Background: Soft tissue sarcomas of the abdominal wall are rare, heterogeneous tumours. The mainstay of management is complete surgical excision.

Unfortunately, recurrence events, are not uncommon. Previously reported prognostic factors include tumour size, depth, grade and surgical margin status.

Purpose: To determine overall survival (OS), disease-free survival (DFS) and factors associated with a poor prognosis in a series of patients, surgically treated for primary abdominal wall sarcomas.

Patients and Methods: Patients undergoing surgical resection of primary abdominal wall sarcomas between April 2008 and May 2018 were retrospectively analysed. Patients were identified from a prospective database in Milan and by retrospectively reviewing clinical computer records in Birmingham. Patient demographics, tumour related characteristics and treatment related characteristics were recorded and analysed. Primary end-points were OS and DFS. Results: 57 patients with primary abdominal wall sarcomas underwent surgical resection.

5-year DFS was 77.5% and 5-year OS was 74.5%. 10/57 (17.5%) patients experienced a recurrence event. DFS was significantly worse in patients with tumours ?5cm (p = 0.012) and in high grade tumours (p = 0.026). Histological subtype was also found to be a significant prognostic factor for DFS (p = <0.001).

Univariate analysis did not demonstrate a significant relationship between tumour depth, type of surgery, surgical margin status or adjuvant therapy and DFS or OS. Conclusions: Recurrence events are not uncommon following the treatment of primary abdominal

Heledd Havard, Royal National Orthopaedic Hospital; Medhi Tofighi, Royal National Orthopaedic Hospital; Craig Gerrand, Royal National Orthopaedic Hospital; William Aston, Royal National Orthopaedic Hospital; Panagiotis Gikas, Royal National Orthopaedic Hospital; Rob Pollock, Royal National Orthopaedic Hospital Tumours of the proximal humerus continue to pose significant surgical challenges both in terms of resection and reconstructive options. The anatomy of the proximal humerus is complex with both capsular and tendon attachments contributing to the biomechanical stability of the shoulder, yet allowing a high degree of functional motion. Resection of tumours or metastases in this area often requires sacrifice of these structures to ensure clear margins and thus necessitates the use of a constrained implant when planning reconstruction.

Early prostheses were associated with high complication rates and poor functional outcome, often serving as little more than a spacer. Evolution of the Bayley-Walker prosthesis in 2006 saw a design modification with the introduction of an O ring - providing additional constraint to the prosthesis.

Despite this adaptation, dislocation remains a recognised complication.

We reviewed 44 Bayley-Walker proximal humeral reconstructions performed in 43 patients. Surgery was performed as a primary procedure in 42 cases with one previously failed nail and one recurrence following curettage. Overall complication rate was 41% consisting of 3 cases of aseptic loosening (7%), 3 infections (7%), 1 wound necrosis (2%), 2 nerve palsies (5%), two periprosthetic fractures (5%) and 7 dislocations (16%). Overall reoperation rate was 16% and overall patient

Page 58

survival was 45%. Of the seven dislocations, only one had resulted in breakage of the O-ring. Five patients underwent revision (proximal humeral body only) with no change in the rotation of the components. Since introduction of the O-ring dislocation rates have reduced from 26% to 16%.

30. Sarcoma UK Support Line - Using patients voices to drive change

Helen Stradling, Sarcoma UK; Sam Hackett, Sarcoma UK; Claire Kelleher, Sarcoma UK The sarcoma UK support line provides support and information to anyone who had questions about sarcoma; be they patients, family members, friends or health care professionals.

Since February 2016 the team have supported 1100 individuals and answered 3800 queries.

The support line gives us a unique view to help us understand the patient perspective. We have monitored trends in queries to identify gaps in service provision, see what we can improve and support the sarcoma community on a local and national level. Support line queries have prompted the expansion of our patient information to cover the diagnosis process, desmoid-type fibromatosis and lymphoedema.

It led to us developing our clinical trials hub which improves access to information on open trials. Questions from patients led to our current work of gaining a consensus on follow up protocols and hormone testing for gynaecological sarcomas. The Support Line puts the patients’ voice at the heart of what we do. It influences the core work of our charity, across information & support, communications, research and policy, to drive us to make choices that have a relevant, positive impact to the sarcoma patient’s journey.

31. Sarcoma follow up survey results

Helen Stradling, Sarcoma UK; Mr Jon Gregory, ROH Birmingham; Dr Paula Wilson, Bristol Sarcoma Team The National Cancer Research Institute (NCRI) clinical studies group for sarcoma has identified follow up of patients following

treatment for sarcoma as a priority for further research. Guidelines regarding frequency of follow up have been in place for a number of years through the British Sarcoma Group (ref 1). From informal inquires it appears that there remain differences in how specialist sarcoma centres follow patients up. Understanding the variation in practice and views of its causes was felt to be important when considering how we develop future trials in this area. A survey monkey questionnaire has been compiled and piloted by a small group the Clinical Nurse Specialists (CNS). The survey consists of 24 questions covering follow up for patients following treatment for sarcoma. Questions cover when/how/and where follow up takes place and also includes questions relating to holistic needs assessments and the Macmillan recovery package. The survey has been sent to all CNSs working in soft tissue sarcoma centres in the UK. The results will be presented and areas worthy of further exploration will be identified and discussed. We hope to gain an understanding of the current practices being undertaken, with thoughts of the next steps in terms of future work.

32. The Prevalence of skip metastases on whole bone MRI for high grade sarcomas of major long bones

Ian Pressney, RNOH; Asif Saifuddin, RNOH Objective: To determine the prevalence of skip metastases diagnosed on whole bone MRI at initial staging in patients with primary high-grade sarcomas of the major long bones.

Materials and Methods: Retrospective review of all patients between January 2007 and December 2017 with a histologically proven high-grade bone sarcoma of the humerus, femur or tibia who had whole bone MRI at initial staging. Data collected included age, sex, tumour type (high-grade osteosarcoma-HGOS, Ewing sarcomaES, high-grade chondrosarcoma-HGCS, dedifferentiated CS-DDCS, dedifferentiated parosteal OS-DDPOS, spindle cell sarcoma-SCS, angiosarcoma-AS and pleomorphic sarcoma-

Page 59

Abstracts - Posters

at two tertiary referral centres

Results: 356 patients were included in the study (males = 199, females =157; mean age 30.8 years, range 4-92 years). Tumour types were as follows: HGOS n=183; ES n=58, HGCS n=39, DDCS n=36, DDPOS n=5, SCS n=23, AS n=6 and PS=6. The overall prevalence of skip metastases was 15.7%. 1% occurred in the humerus, 12% in the femur and 3% in the tibia.

12% were in the same bone, 1% trans-articular and 3% on both sides of the joint. Regarding tumour type, skip metastases were identified in 16% of HGOS, 16% of ES, 8% of HGCS, 31% of DDCS, 0% of DDPOS, 4% of SCS and 17% of AS and PS. Conclusion: Skip metastases are a well-recognised phenomenon in HGOS and ES, having a major impact on surgical management and prognosis.

The current study has also documented the presence and prevalence of skip metastases in other high-grade primary bone sarcomas.

33. The clinical and imaging differentiation of aneurysmal bone cyst and telangiectatic osteosarcoma

Ummesara Zeeshan, RNOH; Ian Pressney, RNOH; Asif Saifuddin, RNOH; Objective: To determine clinical and imaging features which allow a confident differentiation between aneurysmal bone cyst (ABC) and telangiectatic osteosarcoma (TOS).

Materials and Methods: Retrospective review of all patients with a histologically proven diagnosis of ABC and TOS between January 2007 and December 2017.

Data collected included age, sex, site of lesion, radiographic features and MRI findings.

Results: 183 patients were included in the study (males = 92, females = 91; mean age 15 years, range 10-35 years), including 152 cases of ABC

and 31 cases of TOS. 103 (68%) ABCs arose in the long bones and 49 (32%) in the flat bones, while 29 (94%) TOSs arose in the long bones and 2 (6%) in the flat bones. Significant differentiating features between ABC and TOS were bone involved (p=0.001), size (p<0.001), pattern of bone destruction (p<0.001), cortical response (p=0.0001), periosteal response (p=0.0001), Codman’s triangle (p<0.001), soft tissue mass (p<0.001), matrix mineralisation (p<0.001), marrow oedema (p=0.009), and over two thirds fluid-fluid levels (p<0.001). Only soft tissue oedema was not statistically significant between diagnoses (p=0.13).

Conclusion: ABC can be very confidently distinguished from TOS based on multiple radiographic and MRI imaging features.

34. Skeletal Staging of High-Grade Osteosarcoma: A Comparison of Bone Scintigraphy and Whole Body MRI

Ian Pressney, RNOH; Anna Drodz, RNOH; Asif Saifuddin, RNOH Objective: To determine the prevalence of skeletal metastases diagnosed on Tc-99m bone scintigraphy (BSc) and whole body MRI (WBMRI) at initial staging in patients with primary high-grade osteosarcoma (HGOS) of the major long bones.

Materials and Methods: Retrospective review of all patients diagnosed with HGOS of the humerus, femur and tibia between January 2007 and December 2017. Data collected included age, sex, and prevalence of skeletal metastases based on BSc and WB-MRI. Results: 158 patients were included in the study (males = 102, females = 56; mean age 19 years, range 4-70 years). 20 (12%) lesions arose in the humerus, 96 (61%) in the femur and 42 (27%) in the tibia. 75 patients had only BSc performed, 11 patients only WB-MRI and 72 patients had both techniques. The prevalence of skeletal metastases on BSc was 4.7% and

Page 60

on WBMRI was 10.8%. Of the 72 patients who had both tests, 0% of these skeletal metastases were identified only on BSc, in 33% only on WB-MRI and in 66% on both techniques.

Conclusion: Skeletal metastases in HGOS have a prevalence of 4.7% based on BSc and 10.8% based on WBMRI. WBMRI is more sensitive than BSc in the detection of skeletal metastatic disease and can replace BSc.

kurtosis (p=0.012).

Conclusion: MR textural analysis assessing for heterogeneity and in particular higher mean and lower/negative kurtosis can help identify lipoma.

36. Chondral tumours: correlation between needle biopsy and surgical histology

Ines Oliveira, RNOH; Asif, Saifuddin 35. Pilot study to investigate the relationship between lipomatous lesions by MR texture analysis

Ian Pressney, RNOH; Balaji Ganeshan, UCL; Paul O’Donnell, RNOH; Raymond Endozo, UCL Objective: A pilot study to determine the relationship of tumour heterogeneity in lipomatous lesions between MRI texture analysis and MDM2 expression.

Materials and Methods: Retrospective review of 60 patients with a histologically proven diagnosis of atypical lipomatous tumour (ALT) expressing MDM2 and lipoma between December 2015 and May 2018. Data collected included age, sex, site of lesion, MRI findings and textural analysis (TA) of single selected coronal T1 and axial proton density (PD) MRI images that macroscopically contained the most homogenous fat signal.

MRTA comprised a filtration histogram technique.

Results: 60 patients were included in the study; (males = 29, females = 31; mean age years 56, range 27-94 years), including 30 cases of lipoma and 30 cases of ALT expressing MDM2. 8 (27%) lipomas arose in the lower limb, 8 (27%) in the upper limb and 14 (47%) in the trunk and retroperitoneum, while 14 (47%) ALTs arose in the lower limbs, 7 (23%) in the upper limb and 9 (30%) in the trunk and retroperitoneum.

Significant differentiating features between ALT expressing MDM2 and lipoma were age (p=<0.005), size (p=<0.001), medium and coarse texture scales for mean (p=0.003), and

Objective: To determine the accuracy of image-guided core needle biopsy (IGCNB) for the diagnosis and grading of chondral tumours of bone compared to surgical histology.

Materials and Methods: Retrospective review of all patients with a histologically proven chondral tumour of bone who underwent IGCNB between January 2007 and December 2017. Patients with recurrent tumours, incomplete data or who did not undergo surgery were excluded. Data collected included age, sex, skeletal distribution of tumour, technique used for IGCNB, needle biopsy result including histological grade, and surgical histology.

Results: 244 patients were included in the study (males=138, females=106; mean age 60 years, range 13-95years). 179 IGCNBs were CT-guided, 59 ultrasound-guided, and 6 fluoroscopic guided. All 244 (100%) IGCNBs were diagnostic for chondral tumours, although grade could not be determined in 13 (5.3%) due to necrosis. A positive correlation for tumour grade between IGCNB and surgical histology was achieved in 155 (64%). In 68 (28%) IGCNB under-graded the tumour, while in 4 (2%) IGCNB overgraded the tumour. In 2 (1%) diagnosis of dedifferentiated chondrosarcoma was made with IGCNB but surgical histology was diagnostic of mesenchymal chondrosarcoma.

In 2 (1%) IGCNBs were diagnostic of lowgrade chondrosarcoma but surgical histology established the diagnosis of clear cell

Page 61

Abstracts - Posters

PS), and presence of skip metastases based on whole bone T1W SE MRI.

Conclusion: MLS has a high rate of extra-pulmonary soft tissue and bone metastases. This unusual pattern of metastatic spread has been confirmed in this literature review of 970 patients. Note should be made of the relative insensitivity of CT for detecting extrapulmonary soft tissue and skeletal metastases.

Consideration should be given to whole body MRI as well as CT of the chest, abdomen and pelvis for staging MLS.

Conclusion: IGCNB has a very high accuracy for the diagnosis of chondral tumours of bone when undertaken in a specialist sarcoma service.

However, it underestimates the tumour grade in a significant proportion of cases, and therefore should not be considered in isolation of imaging features when planning management.

37. Myxoid liposarcoma – Unusual metastatic pattern and implications for staging and follow-up: A literature review.

38. Staging Investigations Employed in the Diagnosis of Chordomas

Ines, Oliveira; Asif, Saifuddin

Inga Usher, The National Hospital for Neurology and Neurosurgery; Professor David Choi, The National Hospital for Neurology and Neurosurgery; Dr Paul O’Donnell, The Royal National Orthopaedic Hospital

Objective: To determine the prevalence and sites of metastatic disease in myxoid liposarcoma (MLS).

Materials and Methods: Literature review of all major series of MLS describing prevalence and sites of metastatic disease, as well as imaging techniques used for staging and follow-up.

Results: We reviewed the significant literature that has made the greatest contribution to understanding the metastatic pattern of MLS. 10 series were selected, published between 1999 and 2018. The total number of patients was 970 (338 males and 254 females, gender not disclosed in 378 patients) with a mean age of 46 years (range 7-92 years). Metastatic disease was reported in 232 (23.9%) patients, to the lungs in 80 (8.2%) and the extra-pulmonary soft tissue and bone in 200 (20.6%). Several other papers reported a similar trend but details could not be extrapolated. Information regarding the imaging follow-up protocol was only available in some series. However, it is widely reported that when comparing imaging techniques, CT, bone scintigraphy and FDGPET CT significantly underestimated extrapulmonary soft tissue and bone metastases when compared to MRI, which is more sensitive and specific.

Background Chordomas are rare primary tumours of the spine. These tumours affect the axial skeleton and 40% reportedly metastasise. Guidelines based on expert consensus were published in 2015 making recommendations regarding radiological investigations to be undertaken for staging patients, however adoption of the guidelines at centres is variable. We sought to capture current practice at our centre, which is a specialist centre, by documenting the radiological examinations performed in our patient population and whether these yielded positive results.

Methods We identified our cohort of patients with chordoma by searching electronic hospital systems using free text and ICD-10/OPCS4 codes. We then reviewed the records of the patients on our Picture Archiving and Communication System.

Summary of Results We identified a cohort of 42 patients with chordomas. 8/42 (19.5%) underwent whole spine magnetic resonance imaging; none had evidence of a coexistent benign notochordal cell tumour. 7/42 (17.1%) underwent a

Page 62

computed tomography chest scan, revealing metastases in 2 of these 7 patients (28.6%).

Positron emission tomography was performed in 5/42 (12.2%) and a radionuclide bone scan in 4/42 (9.8%) of patients.

Conclusions Although the guidelines recommend magnetic resonance imaging of the whole spine, this was rarely performed at our centre. Chest computed tomography is not recommended in the guidelines but detected chest lesions in a third of our patients in whom the test was employed for specific clinical reasons.

The number of patients who had full staging investigations was low but the yield was relatively high and may influence clinical management.

39. What is a benign notochordal cell tumour? Clinical, radiological and histological characteristics from a large systematic review.

Conclusions BNCTs exhibit a spectrum of features, none of which are pathognomonic and diagnosis is subjective. The majority of reports describe small, non-progressive, non-lytic lesions that lack contrast enhancement, intercellular myxoid matrix and nuclear atypia. They are nevertheless difficult to distinguish from small chordomas.

Inga Usher, The National Hospital for Neurology and Neurosurgery; Professor Adrienne M Flanagan, The Royal National Orthopaedic Hospital; Professor David Choi, The National Hospital for Neurology and Neurosurgery

40. Sarcoma quality performance indicators (QPIs) driving continuous improvements in Scotland

Objectives Clinical, radiological and histological characteristics from a large systematic review.

Design The first systematic review of benign notochordal cell tumours.

Subjects All studies, in any language, published at any time that were identified using specified search terms.

Methods Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).

Results So-called benign notochordal cell tumours exhibited the following features: presentation with pain as the sole presenting symptom

in 35/76 (46.0%) and 34/76 (44.7%) with a neurological deficit. Incidental discovery upon imaging was reported in 7/76 (9.2%).

Radiologically, lesions were small, (<30 millimetres at their maximum diameter) in 91/98 (92.9%), hypointense on T1 sequences and hyperintense on T2 sequences on magnetic resonance imaging (113/133; 85.0%), did not enhance following contrast administration (61/69; 88.4%) and were non-lytic (49/80; 61.3%). Histologically, these tumours lacked myxoid matrix (66/84; 78.6%) and nuclear atypia (100/112; 89.3%). In those managed conservatively, follow up was between 8.5 and 120 months. Most were stable at follow up (22/25; 88.0%), one lesion grew (4.0%), one patient died of an unrelated cause (4.0%) and outcome was not stated in one case (4.0%).

Ioanna Nixon, NHS Glasgow and Clyde; Fiona Cowie, NHS Glasgow and Clyde; Jeff White, NHS Glasgow and Clyde; Dr Larry Hayward, NHS Lothian; Mr Steven Lo, NHS Glasgow and Clyde; Mr Peter Chong, NHS Glasgow and Clyde; Dr Michelle Fergusson, NHS Tayside; Mr David Boddie, Royal Infirmary Aberdeen; Mr Lindsay Campbell, NHS Glasgow and Clyde Better Cancer: Ambition and Action (2016) details a commitment to delivering the national cancer quality programme across NHSScotland, with a recognised need for national cancer QPIs for all tumour types to support a culture of continuous quality improvement. The sarcoma QPIs were implemented for patients diagnosed in 2014/15, refined based on the

Page 63

Abstracts - Posters

chondrosarcoma.

Purpose This study aims to analyse the clinical course of pre-sacral lipomatous lesions, in order to provide evidence as to whether these tumours should be managed as they are currently, according to generic pelvic sarcoma guidelines, or whether they should be considered by guidelines as a separate entity.

Method Data on patient-, tumour- and treatmentrelated variables was collected retrospectively using clinical computer records at a University Hospital Birmingham (UHB). All patients with pre-sacral lipomatous lesions who were referred to the sarcoma MDT at UHB between January 2008 and December 2018 were included in the study. Patients with recurrent lipomatous lesions in the pre-sacral area and patients who had undergone treatment at other centres were excluded from the study.

22 patients were identified for inclusion in this study.

Results Results pending final analysis. Will be available by date of conference

41. Pre-sacral lipomatous lesions – True liposarcomas or co-incidental curiosity?

Conclusion Pending final analysis. Will be available by date of conference

Isabelle Stoddart, University Hospital Birmingham; Hannah Tattersall, University of Birmingham; Max Almond, Anant Desai, Prof David Gourevitch, Samuel Ford

42. Uterine Sarcoma: a single-centre’s experience of treatment and outcomes

Introduction Pre-sacral lipomatous lesions are unusual entities that are frequently referred to sarcoma centres. These rare tumours often come to clinical attention after incidental discovery on cross-sectional imaging. Location deep within the pelvis and size criteria automatically allow for these lesions to be classified as liposarcomas, although in reality such lesions are likely to represent a spectrum of pathological behaviour, which anecdotally is skewed to a more indolent course than liposarcomas encountered elsewhere in the pelvis and beyond.

Ishan Mehta, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust; Han Hsi Wong, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust; Jennifer Harrington, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust; Dochka Davidson, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust; Gail Horan, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust; Helen Hatcher, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust Background:

Page 64

Uterine sarcomas represent a rare group of cancers including: leiomyosarcoma (LMS), endometrial stromal sarcoma (ESS) and adenosarcoma (AS). Classification of ESS has caused confusion over the years, the latest 2014 WHO system has classified these tumours into: low-grade ESS (LGESS), high-grade ESS (HGESS) and undifferentiated uterine sarcoma (UUS). We provide an analysis of our specialist centre’s experience of managing patients with uterine sarcoma, focusing on HGESS and UUS which are associated with poor clinical outcomes.

Methods: Retrospective analysis of treatment and survival outcomes for all patients diagnosed with uterine sarcoma presenting to or under followup by our service between October 2014 to November 2018.

Results: We identified 82 patients with uterine sarcoma during this time period. 52% (43/82) had LMS, 44% (36/82) had ESS and 4% (3/82) had AS. Of those with ESS, 50% (18/36) had LGESS, 14% (5/36) had HGESS and 36% (13/36) had UUS. Focusing on HGESS and UUS, the median age of presentation was 67 and median survival was 18 months (7 months for UUS alone). 44% (8/18) of patients presented with advanced (inoperable or metastatic) disease. For patients with advanced disease, an anthracycline (doxorubicin or epirubicin) was the first-line systemic treatment of choice. In contrast, the median survival for LGESS cannot be determined as only 1/18 patients has died.

More results to follow.

Discussion: Uterine sarcomas are a heterogenous group of diseases. We present our centre’s experience of treating these, focusing on HGESS and UUS which follow an aggressive clinical course with poor outcomes.

43. Head and neck pleomorphic dermal sarcoma – lessons learned from 57 consecutive cases in South Wales.

James Warbrick-Smith, Welsh Centre for Burns

and Plastic Surgery; Johanne Vass, Welsh Centre for Burns and Plastic Surgery; Thomas Bragg, Welsh Centre for Burns and Plastic Surgery Introduction: Pleomorphic dermal sarcomas (PDS) can frequently present to dermatology, maxillofacial or ENT colleagues en route to sarcoma services. There appears to be local uncertainty regarding recommended management. We reviewed cases in South Wales with a view to clarifying and rationalising the MDT management of PDS. Methods: We undertook a retrospective review of cases registered on the Welsh cancer services directory for 2012-16. Pathology and radiology reports were scrutinised together with MDT outcomes.

Results: We identified 57 patients with PDS of the head and neck, 54 of whom were male. Erroneous diagnosis of atypical fibroxanthoma was seen patients outwith the sarcoma MDT. Mean age at diagnosis was 80, with mean follow-up 34 months. 89% of lesions were on the scalp, with most <2cm but some as large as 7cm.

Staging protocols were inconsistent, and in no instance showed metastases at initial tumour presentation. Overall survival was 64%, with 20% of deaths occurring within 6 months, and 46% of deaths within 1 year of diagnosis.

Overall metastatic rate was 13.4%, with patients either having neck nodal metastases or lung and visceral metastases. Recurrence occurred in 3 patients following clear excision margins. Radiotherapy was employed for brain metastases, inner table calvarial erosion, and extra-capsular spread post neck dissection.

Although chest x-rays were not always utilised for follow-up, they identified 2 patients with lung metastases.

Discussion: Our data has informed a pragmatic management pathway for these often frail and comorbid patients. MDT review is mandatory to confirm histopathology of these challenging tumours.

Page 65

Abstracts - Posters

first year’s results and reported for years two and three, reviewed after three years and will be reported for year four in December 2018.

The data is collected locally by each NHS Board and the analysis is performed locally, regionally and nationally with the resultant actions being assigned to the local NHS Board, the sarcoma specialist centre (there are five) or the Scottish Sarcoma Network.

Challenges include assigning TNM stage each sarcoma patient during or before the MDT, reporting molecular pathology in a timely manner, and small number of patients with rare subtypes per year requiring analysis over a 3 to 5 year period. Over 85% of patients with soft tissue sarcoma (SST) extremity sarcoma who undergo surgical resection with curative intent achieved an R0 resection for the first 3 years of reporting and, 91% of patients with SST extremity sarcoma who undergo a planned surgical resection had a histological diagnosis before surgical resection took place by year 3 of reporting compared to 83.6%, 83.1% for year 1 and 2 respectively. Our results will be will be detailed on the accompanying poster/ presentation and demonstrate continuous improvement.

Craig Gerrand, Royal National Orthopaedic Hospital; James Grant, Royal National Orthopaedic Hospital; Tariq Azamgarhi, Royal National Orthopaedic Hospital Background Infection remains a major complication of endoprosthetic replacement of long bones for cancer, including bone and soft tissue tumours.

Treatment routinely includes surgery: options include debridement, one stage and two stage revision, and in recent years the supervision of a specialist bone infection MDT. The aim of this study was to evaluate the demographics of infection and success rates of treatment.

Methodology An observational retrospective study of oncology patients with massive endoprostheses, undergoing surgical treatment of deep infection between 2010 and 2017.

Demographics; tumour type; anatomical site; chemotherapy; radiotherapy; type of surgical intervention; time from initial surgery; organisms from deep tissue sampling; implant type (including silver coating). Patients who had no clinical signs of infection at a minimum of three months follow-up were considered successfully treated.

Results 22 patients were identified as having undergone surgical treatment of deep infection. The following success rates were recorded from the initial procedures; 2/5 twostage, 3/6 single stage, 0/5 debridement and implant retention, 1/2 excision, 2/2 amputation, 1/2 first stage only of two stage revision.

Conclusion In this series one and two stage revision appear to have similar success rates in treating infection, supporting the continued use of single stage for clinically selected patients.

Debridement and implant retention was not successful. Hospital coding systems can be used to identify patients, but may not be

45. Extra-articular resection for Osteosarcoma – Are we getting it right?

James Barnett, Royal National Orthopaedic Hospital NHS Trust; Craig Gerrand, Royal National Orthopaedic Hospital NHS Trust; Asif Saifuddin, Royal National Orthopaedic Hospital NHS Trust Introduction MRI is performed at diagnosis for local staging of osteosarcoma (OS) to aid surgical planning. Where intra-articular tumour is identified, an extra-articular resection (EAR) with endoprosthetic replacement (EPR) is usual but this carries greater surgical risk and higher morbidity. The aim of this study was to assess whether patients undergoing EAR based on pre-operative MRI showing intra-articular tumour had histopathological evidence of tumour in the joint.

Methods Patients were identified from the histopathology database. Those with confirmed histological diagnosis of high-grade OS of the major long bones between 2007 and 2016, and who had undergone EAR with EPR were included.

Results 16 patients met the criteria, 10 males and 6 females with a mean age of 18.7 years (range 11-70 years). The femur was the primary site in 8 cases, the humerus in 6 and the tibia in 2. In 8 cases, there was histological evidence of tumour within the joint in the resection specimen. In a further 7, tumour was noted in the epiphysis or adjacent to the articular cartilage. In 1 case there was no tumour in the peri-articular region. Mortality was 43.8% (7 cases) at a mean of 2.07 years following diagnosis.

Conclusions Histological evidence of tumour within the joint was found in 50% of patients undergoing EAR for OS, which is consistent with previous

Page 66

reports. It is reasonable to consider extraarticular resection when preoperative MRI shows tumour in a joint.

shape future services, only 7% of patients and no relatives/carers responded positively.

Discussion: It was hoped that engaging with patients would inform service development, provide value and reduce inefficiency. The poor response rate needs consideration. Do patients have what they need or do we need a different approach to co-production?

46. “Engaging with adults living with soft tissue sarcoma to inform the development of a new supportive care service – what we learned”

Johanne Vass, Abertawe Bro Morgannwg University Health Board; Hannah Morgan, Abertawe Bro Morgannwg University Health Board; Kathie Wareham, Abertawe Bro Morgannwg University Health Board;

47. Patient preferences in orthopaedic oncology: are patients’ wishes for receiving diagnosis being met?

Introduction: Cancer patient experience surveys suggest that people living with sarcoma do not always feel well supported. In south Wales, there are no sarcoma specific support services outside of the medical/health care system. We aimed to explore what, apart from traditional medical treatment, is important to people with sarcoma, living in a predominantly rural area and what would help them to better cope with their disease and recovery. The information gathered would be used to develop a new supportive care service.

Jonathan Gregory, Hull York Medical School; Emma Ward, Royal Orthopaedic Hospital; Seggy Abudu, Royal Orthopaedic Hospital; Mike Parry, Royal Orthopaedic Hospital; Jonathan Gregory, Royal Orthopaedic Hospital

Methods: Participants were identified using the authors own clinical caseload in conjunction with the Welsh Cancer Network Information System.

Patients and carers were provided with anonymised questionnaires to gather data about their experience of accessing cancer support services. Participants were invited to participate in the planning and development of services.

Purpose To investigate why patients choose a given method of receiving their diagnosis and their satisfaction with this choice in hindsight.

Results: After exclusions, 119 patients were identified.

Two copies of a questionnaire were provided so family/carers could also participate. The overall response rate was 12% (n=29). The response rate for patients was 20% and for carers, 4%. 72% of respondents indicated that they had accessed some form of information or support. 28% of respondents said they would like more sarcoma specific support. When asked if they would like to work with us to

Background Within our service, patients choose how they receive their sarcoma diagnosis: in clinic, over the telephone or by letter. Empirically, most patients select a telephone call. However, it is uncertain why this is the case and whether they are happy with the choice they made.

Methods We recruited patients referred to with a possible diagnosis of sarcoma. Two proformas were used to assess patient preferences, one pre-diagnosis and one for patients postdiagnosis.

Results 65 patients recruited, 26 pre-diagnosis, 39 post-diagnosis. In the pre-diagnosis group, 62% opted to receive results over the telephone, 23% in clinic and 15% by letter.

Wanting to hear back efficiently, being unable to travel back to the ROH and the financial implications of travel were commonly cited for this decision. In the post-diagnosis group, 51% had received their diagnosis in clinic,

Page 67

Abstracts - Posters

44. Success of revision surgery for infected massive endoprostheses as identified from hospital coding

complete. Further prospective study of surgical approaches may be warranted.

Conclusion Most patients received their diagnosis via telephone, predominantly to maximise efficiency and avoid travelling. The majority would not alter their choice with hindsight. Hence, this practice is convenient and popular with patients. A further prospective study to capture how satisfaction with preferences changes through the pathway is planned.

48. Predictors of non-benign lipomas: evidence from a supraregional sarcoma service.

Karishma Khan, Cardiff University Medical School; Mr Thomas Bragg, The Welsh Centre of Burns and Plastic Surgery; Elayne Azzopardi MSc PhD, The Welsh Centre for Burns and Plastic Surgery; Liberto Camilleri PhD, University of Malta Department of Statistics and Operations; Ernest Azzopardi PhD FRCSEd (Plast), The Welsh Centre for Burns and Plastic Surgery Background Suspicion of malignant change within a lipoma is a common and increasing workload within the UK Sarcoma MDT network, and a sourse of considerable patient anxiety. Currently, there is no lipoma-specific data, with regard to which clinical or radiographic features predict nonbenign histology, or calculate an odds-ratio specific to a lipomatous lesion being nonbenign.

Method: A 9-year, double-blind, unmatched cohort study, comparing post-operative histology outcomes (benign versus non-benign) versus 15 signs across three domains: Clinical (size of tumour, depth, growth noticed by patient, previous lipoma, patient felt pain), Ultrasonographic (size, depth, vascular

invasion, heterogenous features, septae) and MRI (size, depth, vascular invasion, heteregenous features, septae, complete fat signal suppression). Reporter Oberver Curve (ROC) analysis, odds ratios and binarly logistic regression analysis was performed doubleblinded on SpSS.

Discussion: This study provides data upon which an informed decision for expedited risk/benefit can be used.

none specifically studying Pelnac®. We aim to describe a case series of the use of Pelnac® to reconstruct sarcoma defects within the East Midlands Sarcoma Service. A Medline search was conducted with search terms: “sarcoma”, “dermal substitute”, “Matriderm®”, “Integra®”, ”Pelnac®”. Data was collected retrospectively over a 2 year period from patient notes and hospital databases. Pelnac® was used in the reconstruction of 4 patients with an average age of 62.5 years. Two patients had myxofibrosarcoma, one had dermatofibrosarcoma protuberans and one had angiosarcoma. The involved anatomical regions were chest (50%) and limbs (50%). The average defect size was 1977cm³, with 50% of excisions resulting in exposed bone. All defects would have required flap reconstruction. All patients had 100% incorporation of Pelnac® and 100% split skin graft (SSG) take after second stage reconstruction. There were no donor site complications. The average duration of surgeries combined was 189 minutes; with the average time from 1st stage Pelnac® application to SSG being 34.5 days. All wounds were mobile and resultant scars were flat and soft in three patients. There was hypertrophic scarring in one patient. Sarcomas require wide excisions resulting in the need for major reconstruction. In our experience Pelnac® is a good reconstructive option for sarcoma defects if autologous reconstruction is not practicable.

49. The use of Dermal Substitute (Pelnac®) after sarcoma excision: a case series

50. Extra skeletal myxoid chondrosarcoma: a monocentric retrospective study.

Muhammad Sarmad Tamimy, Nottingham University Hospital NHS Trust; Lindsay A Shanks, Nottingham University Hospital NHS Trust; Murray G Forsyth, Nottingham University Hospital NHS Trust; Anna Raurell, Nottingham University Hospital NHS Trust; A G B Perks, Nottingham University Hospital NHS Trust; Robert U Ashford, Univeristy Hopsitals of Leicester NHS Trust; Kathryn Steele, Nottingham University Hospital NHS Trust

Louis-Romee Le Nail, Royal Orthopaedic Hospital of Birmingham - Oncology department; Jan Balko Vaiyapur Sumathi, Tomohiro Fujiwara; Jonathan Stevenson, Lee Jeys and Michael Parry

Results With independent consideration, (ROC analysis, followed by binary logistic regression) Ultrasound depth is significant predictor (p =0.044) of a histologically non-benign lipoma.

Ultrasonographically determined vascular invasion and septation were not statistically significant. None of the clinical signs nor MRI were statistically significant (p>0.05). However, heterogeneous MRI features fared better than MRI depth. Ultrasound signs (Pseudo R-Square = 0.105) are more predictive of the post-operation histology outcome than Clinical signs (Pseudo R-Square = 0.082) or MRI tests (Pseudo R-Square = 0.052) Ultrasound and Clinical tests combined (Pseudo R-Square = 0.147) are more predictive of the postoperation histology outcome than MRI tests (Pseudo R-Square = 0.052).

Pelnac® is a dermal substitute which is widely used to reconstruct complex wounds. There is minimal literature documenting dermal substitute use in sarcoma reconstruction and

Page 68

Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare mesenchymal malignant soft tissue tumor characterized by a specific chromosomal translocation. The aim of this study was to analyze general features of this rare sarcoma supposed to be a low grade tumor.

This single referral center retrospective study included 34 patients (25 men, 9 women), with a mean age of 57 years old. All patients were operated on between 1989 and 2017. EMC was mainly deep located (94%) and araised mainly in the buttock or the tight (68%).

Results Mean follow up was 102 months (2-272). Limb salvage was the most common procedure.

However, 4 amputations were required. Twenty patients had adjuvant treatment with 17 cases of radiotherapy and 3 cases of chemotherapy.

Mean event free survival was 71 months (0,7267). The 5 and 10 years overall survival rates were respectively 89 and 68%. Metastasis were mainly to lung and lymph nodes. Mean survival with metastasis was 54 months (3-110). With a minimum of 5 years follow up, risks factors for local recurrence were whoops resection and inadequate margins. Risks factors for metastasis were higher tumor size (11,9 vs 7,7 cm).

Discussion & Conclusion EMC is although usually painless and slow growing tumor. However, it remains a high-grade disease associated to late local recurrence and metastasis. Thus it should be referred to expert center for care. More prognosis factors are needed to better understand tumour behavior and thus carry on with an appropriated treatment.

51. One year Snap-shot of Modern Operative Management of Soft-tissue Sarcomas: The Nottingham Experience.

Lucie Wright, Nottingham University Hospitals; Ciaran Grafton-Clark, University of Leicester; Kathryn Steel, Anna Raurell, Nottingham University Hospitals; Muhammad Tamimy, Nottingham University Hospitals; Robert Ashford, Nottingham University Hospitals Aim: To provide a snapshot of the operative caseload in East Midlands Sarcoma Service (EMSS) with an emphasis on post-operative complications.

Material & Methods

Page 69

Abstracts - Posters

41% by telephone and 8% by letter. This was attributed to: wanting to speak directly with a consultant, avoiding travelling and efficiency.

Retrospectively, 89.7% would choose the same again and 79.4% would elect for the same option even if they lived locally.

Results: 144 patients were diagnosed with a Sarcoma or a related condition in 2017 at EMSS that was managed through the sarcoma MDT; 94 of those patients underwent surgery. 10 of these patients were excluded because they were operated on by another team.

A further 18 were excluded as they were operated on out-with the year of 2017.

Therefore, 66 patients underwent operations in 2017 in our unit. Diagnoses included: leiomyosarcoma (11), undifferentiated sarcoma (11), myxofibrosarcoma (9), ALT (9), other (8), myxoid liposarcoma (6), angiosarcoma (3), dermatofibrosarcoma protruberans (3), dedifferentiated liposarcoma (2), malignant PNST (2), synovial sarcoma (2). The average BMI of patients was 28. 32% had hypertension, 20% were smokers and 12% had diabetes. 18 patients (27%) experienced a complication from surgery including: wound infection (4), haematoma (4), dehisced wound (3), sinus formation (2), partial flap failure (1), seroma (1), persistent high drain output (1), ileus (1), delayed wound healing (1).

Conclusion: The nature of a soft tissue malignancy diagnosis means there is often not opportunity to optimise parameters pre-operatively.

This cohort of patients includes those with comorbidities that may impact on the type of complications arising - this should be taken into account when counselling patients preoperatively.

52. Primary bone tumour patients’ experiences of symptoms prior to diagnosis: a systematic review and qualitative synthesis.

Lucinda Gosling, The Royal Orthopaedic Hospital NHS Trust; Laura Jones, The University of Bimingham; Mike Parry, The Royal Orthopaedic Hospital NHS Tust; Anne Topping

The University of Birmingham Objective: To thematically synthesise primary qualitative studies of the symptom experience of patients with a primary bone tumour, prior to diagnosis.

Design: Systematic review and thematic synthesis Data sources: Searches of Global Health, EBM Reviews, Informit, Cinahl, Web of Science and OvidSP combining terms for patients and relatives; primary bone tumour(s); perception, presentation of symptoms, engagement with HCPs; and qualitative research, were supplemented by searches of specialist journals, guidelines and reference lists. Study inclusion criteria: peer-reviewed qualitative research published in English from 1990 to March 2018; explored pre-diagnosis symptom experience of patients with a primary bone tumour. Of 1545 individual records, only two articles met the inclusion criteria. One paper was based in the UK and one from Taiwan, and reported experiences of 28 primary bone tumour patients. Data extraction: one author extracted data with checking by a second. Data synthesis: synthesising the data of only two studies would potentially be methodologically inappropriate and so a narrative review of the data is in progress, results pending.

Conclusion: This systematic review highlights the lack of primary qualitative studies into the symptom experience of patients with primary bone tumours, prior to diagnosis. It is important that further primary research is undertaken in this area in order to gain an understanding of presentation, diagnostic delay, and ultimately to develop a tool to improve the patient journey and differential diagnosis.

53. MRI of Extra-Skeletal Ewing Sarcoma – a 10 Year Experience

Michael Khoo, Royal National Orthopaedic Hospital; Paul O’Donnell, Royal National Orthopaedic Hospital Introduction: Ewing Sarcoma is a rare tumour which, in

Page 70

the musculoskeletal system, predominantly originates from osseous structures. A small minority originate in soft tissue and represent extra-skeletal Ewing Sarcoma (EES) with a reported incidence in the USA of one per million. We present the MRI features from our 10-year case series.

Methods: All histopathologically diagnosed EES patients were included. Lesion size, location, MRI characteristics and metastatic disease at diagnosis were analysed. Where subsequent follow-up imaging was available, subsequent metastatic disease and local recurrence were assessed.

Results: 15 patients (6M; 9F) with an age range of 14 -70 years were included.

The majority were located in the lower limb (9) with 4 upper limb and 2 central (paraspinal) lesions. Almost all were deep (13) anatomically with 2 superficial. MRI signal characteristics were most likely to be: isointense/intermediate hyperintense T1w signal; hyperintense T2w signal; hyperintense STIR signal; Where IV contrast was administered (11), all demonstrated significant enhancement.

Lesions with necrotic components were in the minority (6).

There was predominantly no surrounding soft tissue oedema (11) and where present (4) this was very limited.

One case had chest metastases at presentation.

7/15 cases had follow-up imaging with one thigh lesion demonstrating subsequent local recurrence. No distant metastases were recorded in these cases.

Conclusion: Extra-skeletal Ewing Sarcoma (EES) is a rarely occurring malignant tumour. We present the most common MRI features from our 10 year case series.

54. A case of delayed response to Olaratumab and remarkable neurological

recovery in metastatic soft tissue sarcoma.

Michael Rowe, Sunrise Oncology Centre, Royal Cornwall Hospital NHS Trust; Benjamin Casey, Sunrise Oncology Centre, Royal Cornwall Hospital NHS Trust; Toby Talbot, Sunrise Oncology Centre, Royal Cornwall Hospital NHS Trust Background Olaratumab can significantly improve overall survival in soft tissue sarcoma when added to doxorubicin and is used first-line in metastatic disease. We describe a case of delayed response to Olaratumab in the context of progressive disease with remarkable neurological recovery from metastatic spinal cord compression caused by a high grade pleomorphic spindle cell sarcoma. Case report A 56 year old female re-presented with metastatic Trojani grade III pleomorphic spindle cell sarcoma following initial radical treatment with surgery and radiotherapy. She was admitted with significant neurological impairment requiring hoist-transfer due to C7 metastatic spinal cord compression and also had numerous pulmonary metastases. Doxorubicin and Olaratumab were commenced but following three cycles imaging demonstrated a mixed response with new pulmonary metastases. Stable disease was achieved after six cycles. The patient then received three cycles of maintenance Olaratumab. Interval CT imaging showed progression of the pulmonary metastases with increasing mediastinal lymphadenopathy and a new pleural effusion. Despite this radiological evidence of progressive disease, her neurological function was improving and three further cycles of Olaratumab were administered based on clinical evidence of response. Repeat CT imaging showed partial response in all sites of previous progression. In addition, her neurological impairment continued to improve, and she was now walking independently.

Conclusion This case suggests there may be a late or delayed response to Olaratumab maintenance treatment via mechanisms that are still poorly understood. Although there was initial radiological evidence of progression, sustained

Page 71

Abstracts - Posters

Methods: EMSS MDT patients operated on in Nottingham City Hospital by the Plastic Surgery team in 2017 were included. Patient notes were reviewed and results analysed using Excel.

55. An audit of re-staging chest CT at first local recurrence of soft tissue sarcoma

Mirza Baig, Barts and the London School of Medicine and Dentistry; Veronica Attard, Royal National Orthopaedic Hospital, Stanmore; Ban Sharif, Royal National Orthopaedic Hospital, Stanmore; Bhavin Upadhyay Royal National Orthopaedic Hospital, Stanmore; Asif Saifuddin, Royal National Orthopaedic Hospital, Stanmore Objective: To determine the prevalence of pulmonary metastases on re-staging chest at the time of first local recurrence (LR) of soft tissue sarcoma (STS).

Materials and Methods: Retrospective review of all patients diagnosed with recurrent STS between January 2007 and December 2017. Data collected included patient age and sex, site of primary STS, time to LR, site of recurrence, initial tumour grade, grade of tumour recurrence, findings of initial staging chest CT, and prevalence of pulmonary metastases on re-staging chest CT. Results: 127 patients were included in the study (males = 80, females = 48; mean age 55 years, range 9 - 92 years). The commonest STSs were Myxofibrosarcoma (32.3%), Spindle cell sarcoma (10.2%), Synovial sarcoma (9.4%), malignant peripheral nerve sheath tumor (MPNST, 8.7%) and pleomorphic sarcoma (6.3%). Initial chest CT demonstrated pulmonary metastases in 4 (3.1%) of cases.

The mean time to LR was 30.8 months (range 3 - 224 months). The prevalence of pulmonary metastases at re-staging chest CT was 22.8%. In 3 patients (10.3%) with pulmonary metastases at STS LR, metastases were also present on initial CT, while in 21 patients (72.4%) there were no pulmonary metastases at initial presentation.

Conclusion:

Re-staging chest CT at the time of first LR of STS identified a prevalence of 22.8% pulmonary metastases, which was highest for grade 3 STS (34.8%), MPNST, pleomorphic sarcoma and synovial sarcoma (range 27.3- 62.5%), which stresses the importance of re-staging chest CT in patients with LR of these STS subtypes.

56. Drug sensitivity testing of soft tissue sarcoma cells at surgery to select personalised chemotherapy: report of a pilot study.

Nicholas C Eastley, University of Leicester; Gareth Griffiths, Imagen Therapeutics; Aurora Sommer, University of Leicester; Ahmed Alhendi, University of Leicester, Joanna DutkoGwozdz, Imagen Therapeutics, Paula McArdle, Imagen Therapeutics, Thomas A McCulloch, Nottingham University Hospitals NHS Trust, Robert U Ashford, University Hospitals of Leicester NHS Trust; Nicola J. Royle, University of Leicester Soft tissue sarcomas (STS) are genetically and morphologically diverse and hence improvement in therapeutic outcomes has been slow. Most primary STSs are treated with curative intent by surgery with neoadjuvant or adjuvant radiotherapy with subsequent regular monitoring to detect recurrence or metastatic disease. Treatment at this stage is often palliative. Additionally, a cohort of patients present with metastatic disease at diagnosis. Given the diversity and rarity of STSs the prospect for the development of novel chemotherapies specifically for STSs is limited.

We have conducted a pilot study to explore the feasibility of testing patient’s STS cells against a range of chemotherapeutic drugs to obtain a drug sensitivity profile for each tumour, in a highly personalised approach. Fresh STS tissue from 22 patients has been disrupted into a single cell suspensions followed by short-term culture to preferentially propagate cancer cells. The STS cells were exposed to therapeutic doses of up to 56 different chemotherapeutic agents, for 72 hours and then screened using a phenotypic assay to directly image and detect cell death. Samples from adjuvant radiotherapy treated STS were

Page 72

mostly not suitable for drug testing, despite the presence of histologically viable tumour in most cases. The 15 samples that were tested successfully showed very variable responses to the tested drugs including Doxorubicin, which is often used in combination therapy for the treatment of metastatic STS. Currently we are using whole exome sequencing to investigate the relationship between genetic alterations in each STS to the profile of drug sensitivities.

57. Preliminary Results of MRI Dixon technique for diagnosis of focal bone lesions

17 cases (45%), the radiograph was unhelpful in diagnosis. Standard MRI allowed a correct diagnosis of a benign lesion in 49 of 55 cases (89%) and a malignant lesion in 20 of 21 cases (95%). The addition of the Dixon sequence changed the MRI diagnosis from benign to malignant in 29 cases (59%) and from malignant to benign in 0 cases (0%).

Conclusion: Addition of the Dixon sequence facilitates the diagnosis of fat containing focal bone lesions but does not aid in the differentiation of benign from malignant bone tumours.

Niels van Vucht, Royal National Orthopaedic Hospital; Asif Saifuddin, Royal National Orthopaedic Hospital; Ian Pressney, Royal National Orthopaedic Hospital; Rodney Santiago, Royal National Orthopaedic Hospital; Bianca Lottmann, Royal National Orthopaedic Hospital

58. Primary Spindle Cell Sarcoma of Bone: Imaging Features in 37 Patients

Objective: To determine the added value of the Dixon sequence for diagnosis of focal bone marrow lesions.

Objective: To determine clinical and imaging features of primary spindle cell sarcoma of bone (1° SCSB).

Materials and Methods: The Dixon sequence was added to the standard MRI protocol for the staging of focal bone lesions presenting to a specialist sarcoma service. Data collected included patient age and sex, lesion site, radiological diagnosis, MRI diagnosis and diagnosis after reviewing the Dixon sequence. All imaging was reviewed by 2 consultant musculoskeletal radiologists, with 3- and 24-years’ experience of bone tumour imaging respectively. Definitive diagnosis was established either by needle biopsy (31 cases) or by classical imaging appearances (45 cases).

Results: 70 patients (36 males, 34 females with mean age 52,6 years and range 7-84 years) with in total 76 lesions were included. At definitive diagnosis, 55 lesions were benign and 21 were malignant. Of the patients who had radiographs, the radiographic diagnosis was correct in 55% and not changed by routine MRI or the addition of the Dixon sequence. In

Nurul A Selamat, Hospital Kuala Lumpur, Kuala Lumpur, MALAYSIA; Asif Saifuddin, Royal National Orthopaedic Hospital NHS Trust, Stanmore

Materials and Methods: Retrospective review of all patients with a histologically proven diagnosis of 1° SCSB between January 2007 and September 2018.

Data collected included age, sex, site of lesion, radiographic features and magnetic resonance imaging (MRI) findings.

Results: 37 patients were included (males = 20, females = 17; mean age 48 years, range 10 - 85 years).

Thirty-two lesions (86.5%) arose in the long bones, of which 27 (73.0%) involved the lower limbs, while 4 cases (10.8%) arose in flat bones and 1 (2.7%) in the spine. Radiographs were available in 30 cases (81.1%), in which 5 cases (16.7%) showed a non-aggressive pattern of bone destruction while 25 cases (83.3%) showed an aggressive pattern of bone destruction. Matrix mineralisation was seen in only 1 case, while 8 cases demonstrated cortical destruction. MRI demonstrated nonspecific signal characteristics of solid soft-tissue mass, with only 2 cases demonstrating small

Page 73

Abstracts - Posters

treatment led to objective clinical and radiological response and significant, ongoing neurological recovery.

Conclusion: 1° SCSB should be considered in adult patients presenting with an aggressive lytic lesion without matrix mineralisation, especially cases involving the long bones with large intramedullary and extra-osseous solid softtissue components.

59. Retrospective Review of free fibula transfer in paediatric sarcoma: A single Centre Study of epiphyseal transfers

Paul Roblin, Guy’s & St.Thomas Hospital, Londo, UK; Maleeha Mughal, Toronto General Hospital, Toronto, ON, CA; Victoria Rose, Guy’s & St.Thomas Hospital, London, UK; Robin Pollock Royal National Orthopaedic Hospital, Stanmore, London, UK INTRODUCTION: The concept of skeletal reconstruction in paediatric bony sarcoma encompasses replacement of tissue defects and the potential for osseous growth. The free vascularised Fibular graft provides rapid autograft integration and the potential for a physeal transfer in paediatric patients. We present our experience in management of paediatric skeletal reconstruction with the Fibular flap.

METHODS: A retrospective review of all paediatric extremity cases undertaken at our unit by the senior authors was carried out. Data collection parameters included patient demographics, diagnosis, tumour location, size of resection, size of skeletal reconstruction, flap ischaemia time, vessels used for anastomosis, postoperative complications, time to union, time to ambulation/mobilisation and measurement of limb growth.

RESULTS: A total of 6 cases were reviewed with a mean age of 5.8 years. Four male and two female patients were identified.83.3% had neoadjuvant chemo and radiotherapy. All patients underwent epiphyseal and diaphyseal transfer with a dual anastomosis utilising the recurrent branch of anterior tibial artery for the physeal transfer. Rate of primary union at six months follow up was 100% with an annual longitudinal growth of 17mm on average. Complications included a periprosthetic fracture following trauma which was treated conservatively and a donor site wound dehiscence.

CONCLUSION: Enhanced microsurgical and oncological advances have resulted in improved prognosis and survival rates in paediatric sarcoma.

In addition, limb salvage surgery has been associated with a more prolonged survival than the historically accepted primary amputation.

In the younger patient skeletal reconstruction post resection should meet demands of high function and cosmesis.

60. Dosimetric characteristics, outcomes and prognostic factors of patients from University College London Hospitals (UCLH) with extracranial chordomas and chondrosarcomas treated with high dose radiotherapy.

Pei Shuen LIM, Paul Scherrer Institute, University College London; Mahbubl Ahmed, University College London Hospitals; Franel Le Grange, University College London Hospitals; Danny Indelicato, University of Florida Health, USA; Damien Weber, Paul Scherrer Institute, Switzerland; Beatrice Seddon, University College London Hospitals Chordomas and chondrosarcomas are rare bone malignancies which are locally invasive.

We report the demographics, dosimetric characteristics and prognostic factors of patients from UCLH who received high-dose radiotherapy(>45Gy EqD2) for extracranial chordoma and chondrosarcomas between December 2002 and October 2017. 72 patients with chordomas(n=43) and

Page 74

chondrosarcomas(n=29) were included. The median age at radiotherapy was 54.3 years.

Median follow-up was 21.7 months. 44% received proton and 56% received photon radiotherapy. The median dose of prescribed and received radiotherapy (PTV-D95%) was 70.1Gy and 60.5Gy to the high-risk area; 49Gy and 50.7Gy to the intermediate-risk area. The median PTV-HD-D95% high-dose received by type of radiotherapy plan was 67Gy(proton), 58.5Gy(mixed proton/IMRT), 61.6Gy(IMRT) and 47.7Gy(3D-CRT). The 5-year local-recurrence-free survival(LRFS), distant-metastases-free survival(DMFS) and overall survival(OS) rates were 40%,73% and 50% in all groups; 24%,58% and 21% for chondrosarcoma; 54%,85% and 75% for chordoma. LRFS and OS were significantly influenced by histology, grade and tumour size at diagnosis. The 4-year-LRFS was greater when PTV-HD volume<850ml(57%vs18%), PTV-HD-D98%?67Gy(100%vs38%), PTVHD-D95%?67Gy(92%vs32%) and PTV-HDD80%?70Gy(82%vs31%). 4-year-OS was affected by type of radiotherapy(proton 92% vs photon 45%) and status of local disease at time of radiotherapy(none/microscopic 70% vs gross 24%). Local progression postradiotherapy had a significant impact on 4-year-DMFS(100%vs40%) and 4-yearOS(79%vs50%), highlighting the importance of local control. In this subgroup of patients who experienced local progression, OS was prolonged by any salvage intervention (4-yrOS 69%v30%). These results indicate that high-dose radiotherapy with good qualityassured dosimetric coverage is fundamental in the treatment of patients with extracranial chordomas and chondrosarcomas.

Aim: Review the patients who present very late relapse beyond 10 years after the primary surgery.

61. Very Late Relapse In High Grade Soft Tissue Sarcoma In Extremities

Robert Grimer, Royal Orthopaedic Hospital, Birmingham; Michael Parry, Royal Orthopaedic Hospital, Birmingham

Qi Yin, Royal Liverpool University Hospital; Tim Helliwell, Royal Liverpool University Hospital Patients with high grade soft tissue sarcoma, 80% of relapses occur within the first 2 years after definitive resection. However, relapse after 10 years is uncommon.

Methods: We present five cases with high-grade soft tissue extremity sarcomas who had very late relapse from 11 to 29 years after primary local surgery and followed by radiotherapy. Histologically, two synovial sarcomas, two myxoid fibrosarcomas and one leiomyosarcoma. These patients were strictly followed up in the sarcoma service as per sarcoma protocol and discharged at 10 years after primary surgery. These patients were re-referred urgently back to sarcoma service with signs of local recurrence and metastatic diseases after 1 to 19 years following discharge.

Results: The images have demonstrated metastatic deposits in all and local recurrence in two cases. All were confirmed on histology following biopsy. Two patients had further surgery (removal of soft tissue metastatic lesions) and three patients were referred for palliative chemotherapy/radiotherapy.

Conclusion: Late relapse is not common in sarcoma, but can occur in some subtype of sarcoma.

Therefore, we suggest prolonged surveillance or life-time follow up for certain subtypes of sarcomas such as synovial sarcoma and myxoid fibrosarcoma.

62. What is the significance of a ‘whoops’ procedure? A review of the world literature.

Aim: To review the world literature on ‘inadvertent excision (IE)’ of lumps that then turn out to be soft tissue sarcomas and identify if there is consensus on management and what effect IE has on outcome.

Page 75

Abstracts - Posters

internal cystic components manifest as fluid levels. The mean length of marrow involvement was 100.4 mm (range 15 - 253 mm). Skip metastases in the same long bone were seen in 4 (10.8%), and an extra-osseous mass was demonstrated in 32 (86.5%). Lung metastases were diagnosed during staging of the disease in 9 (24.3%) patients.

Results: 39 papers have provided useful information on either the incidence, management or results of patients following IE of a STS. These papers date from 1985 to 2018 and contain details of over 3000 patients. The incidence of IEs varied from 18 to 53% of all patients presenting with STS and was more common in superficial rather than deep sarcomas but is decreasing with time. The median size averaged 5.7cm.

Treatment was with wide re-excision followed by radiotherapy. Residual tumour was found in 50% of cases and the presence of this was a poor prognostic factor for both survival and local recurrence. There was some evidence that radiotherapy before re-excision had better outcomes in terms of local control. Overall survival did not seem to be worse following a previous IE but there was a higher rate of patients needing flaps and amputation. The total cost of treatment was almost doubled by having a previous IE. Conclusion: Inadvertent excision of lumps which turn out to be STS can be decreased by education.

Wide re-excision and radiotherapy remain the best treatment. There may be a place for a clinical trial comparing pre vs post operative radiotherapy in this situation.

63. Two-stage surgery for myxofibrosarcoma: Resect & VAC

Robert U. Ashford, East Midlands Sarcoma Service; Kathryn H. Steele, East Midlands Sarcoma Service; Anna Raurell Rosique, East Midlands Sarcoma Service; Thomas A. McCulloch, East Midlands Sarcoma Service; M. Sarmad Tamimy, East Midlands Sarcoma Service Myxofibrosarcoma is a challenging tumour to manage surgically. Not infrequently it has infiltrative margins, not always visible on cross sectional imaging and surgical margins can be suboptimal. This can result in reconstructions

after resection being compromised by either requiring to be taken down or alternatively by adjuvant radiotherapy It has been our practice in recent years to primarily resect myxofibrosarcomata and then to VAC the surgical wound pending margin status. At the first procedure a reconstructive surgical plan will be determined. If the surgical margins achieved are clear, the reconstruction proceeds as planned mindful of plans for adjuvant radiotherapy. If however the margins reveal atypical or sarcomatous cells then a further resection is undertaken without compromise of any soft tissue reconstruction. We present illustration of the technique and our early outcomes. We believe this techniques optimises both surgical and reconstructive outcomes.

64. Long read nanopore sequencing of well differentiated retroperitoneal liposarcoma (WD-RPLS) reveals recurrent mutations in FOXD4L3 gene.

Robert Tyler, Queen Elizabeth Hospital; Dr. Jo Stockon, Institute of Cancer and Genomic Sciences, University of Birmingham; Mr. Anant Desai, Department of Sarcoma Surgery, Queen Elizabeth Hospital; Dr. Andrew Beggs, Institute of Cancer and Genomic Sciences, University of Birmingham Introduction A large number of sarcomas have been found to have consistent chromosomal abnormalities. Previous comprehensive multi-platform molecular characterisation of WD-RPLS at our institution revealed a recurrent mutation in FOXD4L3, a transcription factor. Our aim was to validate this mutation in a complex area of the genome by generating long reads using nanopore technology.

Materials and Methods 13 samples (1 cell line, 4 tumour: normal, 4 tumour only) underwent long range PCR of DNA using custom primers for FOXD4L3. The PCR products then underwent long amplicon (2.5kb) nanopore sequencing to an average of 4500x read depth using the Oxford Nanopore Technology Minion platform. Amplicon data

Page 76

was then mapped to the reference genome.

Results Long read nanopore sequencing revealed that 8/8 tumours had detectable mutant reads at FOXD4L3 (VAF range 0.17-0.59) compared to normal samples (0.0-08.) A commercially available cell line (CRL-3043), representative of WD-RPLS also confirmed the mutation.

Conclusion We have successfully used long read nanopore sequencing to validate the FOXD4L3 mutation in a complex area of the genome. Interrogation of the literature suggests FOXD4L3 may interact with TP63, a well-recognised tumour suppressor gene and therefore play a role in tumourigenesis. Nanopore technology expedites the resolution of challenging genomic regions, commonly found in sarcoma samples. To our knowledge, this is one of the first ventures into nanopore sequencing in sarcoma, and the first in liposarcoma. Follow up work now aims to explore the role of FOXD4L3 in tissue models.

65. Ten year follow- up of patients with soft tissue sarcoma; East Midlands experience.

Anna Raurell, Nottingham University Hospitals; Rossel Morhij, Nottingham University Hospitals; Sarmad Tamimy, Nottingham University Hospitals; Kathryn Steele, Nottingham University Hospitals; Rob Ashford, Leicester Royal Infirmary Introduction: Soft tissue sarcoma (STS) is a rare tumour with high local, regional and mortality rate.

Five-year survival rates for those diagnosed with STS have been reported as low as 50% in some centres. The purpose of this study was to assess the local, regional and mortality rate in the region for patients undergoing soft tissue sarcoma resection with curative intent.

Method: Retrospective study of patients who underwent potentially curative resection of soft tissue sarcoma in 2008 in East Midlands. All patients’ records, including pathology reports and

clinical notes were reviewed. Data regarding demographics, status at presentation, adjuvant therapy and tumor characteristics (histology, pathology, grade, site, size, surgical margins) were identified.

Results: 54 patients were identified. 8 (15%) were lost in follow up. The majority of patients were males 60%, mean age 63. Majority of tumours were greater than 10 cm (57%), 95% were high grade. All patients had min 10 years follow up. 10% developed local recurrence, 63% developed distant metastasis. 60% died of their cancer median survival was 34 months, range (4-84 months).

Discussion: Our mortality, and local recurrence rate is similar to published data. The majority of STS relapses occur within 5 years after treatment. our study shows that late disease recurrence among patients with STS is not uncommon.

66. Metronomic cyclophosphamide and prednisolone in patients with pre-treated metastatic sarcoma: a single centre retrospective audit

Ruhi Kanani, University College London Hospital; Anne McTiernan, Palma Dileo, Beatrice Seddon, Jeremy Whelan; Sandra Strauss, University College London Hospital Background: Data is limited on the use of metronomic cyclophosphamide and prednisolone (CP) in metastatic sarcoma patients; particularly in those who have received prior chemotherapy.

Methods: We conducted a single centre retrospective audit of sarcoma patients treated with CP between February 2012 and October 2018.

Patients were radiologically evaluated according to standard of care, most commonly after two or three cycles of treatment. Clinical benefit (CB) was defined as lack of clinical or radiological progression.

Results:

Page 77

Abstracts - Posters

Method: A literature review of all papers dealing with IE of sarcomas and the data summated.

Conclusion: This retrospective audit of metronomic CP in heavily pre-treated sarcoma patients demonstrates only limited benefit and does not support routine use. Analysis is ongoing to identify patient groups most likely to benefit to inform future care.

67. A survey to investigate current local practice in the UK for the testing of hormone receptors in ULMS /ESS and the impact this result has on whether HRT is considered.

Sam Hackett, Sarcoma UK; Charlotte Benson, Royal Marsden Hospital; Malee Fernando, Sheffield Teaching Hospital; Sarah Pratap, Oxford University Hospital.

Introduction Sarcoma UK provides a support line run by two specialist nurses and a support and information lead. In March a caller to the line asked whether Uterine Leiomyosarcoma (ULMS) should be routinely tested for hormone receptors, and if the tumour was then found to be ER/PR negative, if HRT would be a possibility. In trying to answer this question we realised there was a difference in practices taking place across the country.

Methods: In collaboration with the NCRI Sarcoma Clinical Studies Group, Gynae Sarcoma Sub Group and input from the NCRI Gynae CSG, we compiled a short questionnaire to investigate further for both ULMS and Endometrial Stromal Sarcoma. Included in the survey are questions on how the pathology staining is expressed, whether the hormone status impacts treatment decisions and if the tumour is ER/PR positive could HRT still be offered. The survey is still ongoing and we would like to provide the responses to date and use the opportunity to promote further engagement at the BSG conference.

Conclusion: We hope that the results of the survey may provide a platform to debate the issues further to form some consensus, provide an opportunity for potential research and ultimately answer the question for the support line caller.

68. Sarcoma Limb Immobilisation

Samantha Farrington, Mark Edwards, Rhys Jenkins, Douglas Etheridge, Colin Craven, Ada Li, Department of Medical Physics & Clinical Engineering, Singleton Hospital Swansea; Dr Kath Rowley, Department of Clinical Oncology and Radiotherapy, Singleton Hospital Radiotherapy plays an important role in the multi-disciplinary management of adult soft tissue sarcomas. Intensity Modulated Radiotherapy (IMRT) and Volumetric Arc Therapy (VMAT) are now the preferred treatment modalities for a number of tumour sites where highly conformal dose distributions can be delivered to the target, with a sharp falloff in dose to the surrounding tissue to reduce the risk of toxicity. In order to treat Sarcoma patients with these advanced techniques, the department was keen to improve the current immobilisation method of relying solely on vac-bags, as they tend to denature over the weeks of treatment from the patient climbing in and out of the bag, and it is difficult to reproduce the daily set-up and align the

Page 78

tattoos and reference marks. The Mould Room has designed and developed a technique using in-house materials such as thermoplastics, single-use patient specific moulding materials and clear Perspex to immobilise the limb with a higher degree of accuracy and reproducibility.

The main aims were simplicity, ensuring patient comfort and to provide an immobilisation system for the Radiographers to use in the CT-Sim and the Treatment Units which was user-friendly, quick to immobilise the area and provide a platform for a be-spoke set-up for each patient so that referencing and essential information can be written onto the baseboard and accessories to enable greater efficiency and accuracy in the daily set-up. Developing an in-house solution enables the design to be adapted as further experience is gained, while keeping costs low. Initial trials and audits were undertaken to determine the best way to use the materials and obtain the accuracy and reproducibility we were looking for. To date we have treated seven patients using this immobilisation technique and have had good results with daily set-up, verification and positive feedback from the Radiographers. We are now further developing the immobilisation by using a ‘popper’ system of fixation similar to that used for head and neck masks to further increase accuracy and reproducibility. We are aiming to treat our first IMRT/VMAT sarcoma patient in April 2019 after the immobilisation system has been tested and the reproducibility validated for conventional treatment techniques.

69. Is it time for a national sarcoma database?

Sandra McAllister, Morriston Hospital; Robert Slade, Morriston Hospital; Thomas Bragg, Morriston Hospital Patients with soft tissue sarcoma (STS) are managed within multidisciplinary teams.

The limitations of information management systems means that it can be difficult to collect data across the different specialties and hospitals where patients receive care. Recently, a new database has been designed by the team, to capture detailed information about

the demographics, diagnostic procedures, histopathological diagnosis, surgery and additional treatments, along with key quality identifiers to determine the standards of service provision. The aim is to prospectively collect robust data across the South West Wales Cancer Network. As a pilot project to test the usability of the data collection method, a retrospective review has been undertaken of the care of patients with soft tissue sarcoma, managed in the South West Wales Cancer Network. Results of the above data points will be discussed, along with survival data for 1-, 3- and 5-years post diagnosis. Quality of care will be determined against the predetermined standards for the service. The aim is to continue with prospective data collection in this geographical area. In addition, the discussion will include the possibility and potential to develop a facility to collect data on a national level, for collaborative research.

70. Why Having the Right Numbers Count

Sarah McDonald, Sarcoma UK; Bevis Man, Sarcoma UK As the national charity for all sarcomas, Sarcoma UK recognises the need for accurate, up-to-date information and data about the cancer. There hadn’t been new incidence figures since 2009 for sarcoma, and 2011 for bone sarcoma. Knowing this would not only give a fairer representation of the scale of the issue and the shape of the patient cohort, it could also influence decisions made for our supporter community. In Autumn 2017 we set about finding out updated UK incidence and survival data for sarcoma. This proved to be a steep learning curve for us, covering many areas; data collection at Trust / CCG level, role of NCRAS and PHE, four nations with different data sets, cohort size and information governance. When we got the data, we then looked at what it told us about sarcoma incidence and survival. The lack of depth of data available and what a limited picture it gave was very frustrating. Looking at it highlighted a number of issues relating to not just data but coding as well. We then took a step back from the figures, one of

Page 79

Abstracts - Posters

Eight-six patients, median age 61 years (range 7-83), with metastatic sarcoma were included.

Median time from 1st line chemotherapy to CP was 14.5 months (range 0-107). Median number of prior regimens was 2 (range 0-7); 20 patients (23%) had received >4 lines of therapy. Performance status (PS) was 0 -1 in 26 patients (30%), PS >2 in 25 pts (29%) and unknown in 35 patients (41%). Fifteen patients (17%) had CB with a median progression-free survival of 4.5 months (range: 1.5-34 months) including 4 who remained on treatment for > 1 year. Forty-three patients (50%) progressed at first radiological assessment and 28 were discontinued prior to first assessment due to death (n=14), clinical progression (n=4), or poor tolerance/toxicity/choice (n=7). Median PFS for whole cohort was 2 months (range: 0-37). Median overall survival was 3.5 months (range: 0-55).

71. Management of primary soft tissue spinal sarcomas at the Royal Marsden Sarcoma Unit 2000-2017

Dr Emma Burke, The Royal Marsden Hospital; Dr Aisha Miah, The Royal Marsden Hospital; Dr Henry Mandeville, The Royal Marsden Hospital; Dr Frank Saran, The Royal Marsden Hospital; Dr Shane Zaidi, The Royal Marsden Hospital

Gy(range 50.1-64 Gy), median duration 41 days (range 35-45 days). Median time to radiotherapy following diagnosis 174 days (range 22-272 days). Median follow-up at RMH was 2.9 years. Median time to local (n=4) and metastatic (n=3) recurrence were 2.9 and 5.9 years respectively. Median OS 4.3 years (5/11 patients died).

Analysis: Rarity and heterogeneous histology make primary spinal soft tissue sarcomas challenging to treat, as reflected in the variety of techniques used in these cases.

Clear guidelines, with consideration of new techniques including proton therapy, are needed to improve outcomes.

72. Developing a holistic personalised model of rehabilitation care to optimise outcomes after treatment for lower extremity sarcoma.

Aims: There are no consensus guidelines on the management of soft tissue spinal sarcomas.

This study determines the management and outcome of patients with primary soft tissue spinal sarcoma treated in the RMH Sarcoma MDT between 2000-2017.

Methods: A retrospective analysis of patients was undertaken from prospectively maintained database and electronic patient records.

Histologically proven primary soft tissue sarcoma patients treated with radical intent were included.

Results: 11 cases (6 males and 5 females) with a median age of 41 (range 14-67) were identified.

Disease location was categorised as cervical (n=2, 18%), thoracic (n=4, 36%) or lumbar (n=5, 45%). Histological subtypes were Ewing’s (n=5, 45.5%), Chondrosarcoma (n=3, 27.3%), spindle cell (n=1, 9.1%), angiosarcoma (n=1, 9.1%) and haemangiopericytoma (n=1, 9.1%). Treatment modalities were surgery, radiotherapy and chemotherapy (n =5, 45.5%) or surgery and radiotherapy (n=5, 45.5%) or chemotherapy and radiotherapy (n=1, 9%).

Patients received radiotherapy using virtual simulation (n=1), conformal (n=3), IMRT(n=6) or proton beam (n=1). Median dose 59.4

Dr Sherron Furtado, Royal National Orthopaedic Hospital, Stanmore, London, UK; Dr. Silvia Del Din, Institute of Neuroscience/ Newcastle University Institute for Ageing, Clinical Ageing Research Unit, Campus for Ageing and Vitality, Newcastle University, Newcastle upon Tyne, UK.; Dr Jan.lecouturier, Institute of Health & Society, Newcastle University, UK.; Mr Kenneth Rankin, Dept. of Orthopaedics, North of England Bone and Soft Tissue Tumour Service, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; Professor Lynn Rochester, Institute of Neuroscience/Newcastle University Institute for Ageing, Clinical Ageing Research Unit, Campus for Ageing and Vitality, Newcastle University, Newcastle upon Tyne; Mr Craig Gerrand, Royal National Orthopaedic Hospital, Stanmore, London, UK Background: Treatments for sarcoma lead to a wide range of impairments and participation restrictions, however rehabilitation services after sarcoma are highly variable. Taking a personalised holistic evidence-based approach towards rehabilitation delivery might significantly target service gaps. The aim of this study

Page 80

was therefore to assess the feasibility of developing holistic personalised rehabilitation plans (PRPs) using a structured rehabilitation problem-solving (RPS) assessment [based on the International Classification of Functioning, Disability and Health (ICF)].

Methods: Patients had an RPS assessment using impairment (MSTS), disability (TESS), quality of life (QoL-CS) scales, interviews, an instrumented testing of balance, gait and timed up and go (iTUG) in the clinic and 7 day community monitoring (using Axivity AX3).

Domains of the RPS map to interventions commonly used in clinical practice (e.g: impaired balance ? balance rehabilitation, reduced physical activity levels ? activity reminders). PRPs were developed, by selecting these interventions, to target domains affected in their RPS assessment.

Results: 11 adults were enrolled. Interim analysis revealed that it is feasible to perform RPS assessments and develop PRPs for patients.

This approach captured patient preferences about their rehabilitation care. A combination of different domains of physical function (balance, gait, disability, physical activity, QoL) were affected after sarcoma. PRPs required refinement at various time points (due to complications/ treatment progression).

Conclusion: Preliminary findings support the feasibility of performing RPS assessments to develop PRPs for patients after sarcoma. Each PRP requires to be refined during patient’s clinical journey. This evidence-based personalised model of care promotes delivery of patient-centred care.

73. A markerless motion capture to quantify functional outcomes using a depth-sensor after treatment for lower extremity sarcomas in the clinic: A feasibility and validity study.

Sherron Furtado, Royal National Orthopaedic Hospital, Stanmore, London, UK; Dr Brook Galna, Institute of Neuroscience, Newcastle

University, Newcastle Upon Tyne, UK; Dr Alan Godfrey, Computer and Information Science, Northumbria University, Newcastle Upon Tyne, UK; Mr Craig Gerrand, Royal National Orthopaedic Hospital, Stanmore, London, UK. Background: Physical limitations are widely reported after sarcoma, yet rapid quantitative functional assessments are lacking. Expensive cumbersome systems pose a barrier to clinical translation. Our aim was therefore to assess the feasibility and validity of a single portable inexpensive markerless motion capture sensor (Microsoft Kinect) for functional assessments after lower extremity sarcoma treatments.

Methods: Motion capture of three standard activities; single-leg stance, stand to kneel/kneel to stand and normal pace walks were quantified using Kinect. Summary measures of balance [AP and ML range during single-leg stance and AP, lateral range and movement velocity during kneeling] and gait [step length and velocity] were derived. Kinect measures were compared to existing measures [disability scale (Toronto Extremity Salvage Score (TESS)) and impairment scale (Musculoskeletal Tumour Rating System (MSTS)] to establish convergent validity.

Results: 34 adults of mean age 43 (19-89) years recruited, included patients treated for tumours in the femur (19), pelvis/hip (3), tibia (9), or ankle/foot (3). 27 had limb sparing surgery and 7 amputation. Motion capture using Kinect were well tolerated and produced clinically useful data with face validity. Significant correlations were seen between (i) MSTS and balance (p=0.007*, r=-0.567), movement velocity (p=0.020*, r=0.502) and gait (p=0.022*, r=-0.416); and (ii) TESS and balance (p=0.015*, r=-0.564) (Figure 1) and movement velocity (p=0.021*, r=-0.541).

Conclusion: This study supports the feasibility and validity of using a single depth-sensor to quantify physical functioning in sarcoma patients.

Structural impairments are significantly

Page 81

Abstracts - Posters

which suggested that sarcoma incidence had increased from 3800 to 5400, and looked at what this meant for the sarcoma community.

Did it make sarcoma more or less rare? A key factor for us was increasing awareness of sarcoma and having these figures helped us shape new key messages to use, and what we want to do next.

74. The route to diagnosis (RtD) of sarcoma patients: a qualitative study in the Netherlands and the United Kingdom

V.L.M.N. Soomers, Radboudumc; I.M.E. Desar, Radboudumc; S. Zaidi, Royal Marsden Hospital NHS Trust; A.J. Hayes, Royal Marsden Hospital NHS Trust; W.T.A. van der Graaf, Royal Marsden Hospital NHS Trust; O. Husson, ICR London Objective Sarcomas are a rare group of mesenchymal tumours that have considerable heterogeneity with respect to age of onset, presentation, anatomic location, tempo of progression and outcome. Early and adequate diagnosis is difficult, but important for local control, survival and quality of life. In order to improve the route to diagnosis (RtD) it is important to understand sarcoma patients’ appraisal, helpseeking behaviour and the diagnostic process.

Until now, limited knowledge is available on the sarcoma patient experience of the RtD. The aim of this qualitative study was to examine the experienced diagnostic pathway and factors that contributed to the duration of the RtD from a patients’ perspective.

Methods In preparation of a larger multicentre quantitative study we conducted semistructured interviews with sarcoma patients diagnosed within the past 4 months in one of the participating sarcoma centres in The Netherlands (Radboudumc, Nijmegen) and the United Kingdom (Royal Marsden Hospital, London). The RtD was defined as the time of the first symptoms until the time the patient was informed of the pathological diagnosis.

The interviews were analyzed according to qualitative content analysis. Apart from topics derived from the data, we used predefined topics derived from a literature review. These were based on Walter’s Model of Pathways to Treatment, which provides a framework for research of the complex and dynamic nature of the RtD. The last interval, pre-treatment,

falls beyond the scope of our research and experiences were thus attributed to one of three categories: (1) appraisal, (2) help-seeking, and (3) diagnostic. Appraisal refers to the time interval between detecting bodily changes and help-seeking of a healthcare provider (HCP), whereas help-seeking is the period between the decision to consult a HCP until the actual appointment. The diagnostic phase refers to the period from diagnostic tests to histological diagnosis.

as well. This study lays the groundwork for future research into improving RtD for sarcoma patients. Quantitative, prospective studies are needed to quantify TtD, examine risk factors for prolonged TtD and assess the impact on clinical and patient-reported outcomes.

Results Seven Dutch and eight English patients with a time to diagnosis (TtD) varying between 10-145 weeks participated. They reflect the heterogeneity of the population. The experiences of patients with regard to RtD were categorized according to Walter’s Model.

In all cases with a long TtD, patients were not aware their symptoms could be caused by a malignant diagnosis. Themes that prolonged appraisal were (1) alternative explanations for bodily changes; (2) normalising symptoms; (3) other life priorities. Triggers for help-seeking were (1) interference of symptoms with daily life; (2) persistence or increase of symptoms.

Two types of patients could be identified during the help-seeking interval: those who received diagnosis after first HCP consultation and those who experienced repeated cycles of appraisal and help-seeking. In this last group reassurance without a safety-net, absence of a lead HCP, or HCPs who did not start a new cycle of clinical reasoning on 2nd request were factors that prolonged the TtD. During the diagnostic phase patients mentioned waiting times as the factor contributing most to a long TtD. Waiting caused distress, decreasing the quality of life.

V.L.M.N. Soomers, Radboudumc; O. Husson, ICR London; D.C. Strauss, Royal Marsden Hospital London; R.J. Young, Weston Park Hospital, Sheffield, United Kingdom; M.C. Parry, Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham UK; W.T.A. van der Graaf, Royal Marsden Hospital London; M.G. Leahy, The Christie NHS Foundation Trust, Manchester U.K; A.J. Hayes, Royal Marsden Hospital M.J. Smith, Royal Marsden Hospital; L.V. van de Poll-Franse, Tilburg University The Netherlands and Netherlands Comprehensive Cancer Organisation, Utrecht The Netherlands I.M.E. Desar, Radboudumc

Conclusion Three phases of the RtD were identified ((1) appraisal, (2) help-seeking, and (3) diagnostic), all with room for improvement to decrease TtD. This study suggests that awareness of patients that their symptoms may be caused by something serious could accelerate appraisal and help-seeking, and awareness of HCPs about the possibility of sarcoma could shorten the diagnostic and pre-treatment phase which could reduce distress due to waiting times

Page 82

75. The QUEST study: assessing the QUality of life and Experiences of Sarcoma Trajectories: study protocol

Background The prognosis of patients with sarcomas suffers from delay in diagnosis. Routes to diagnosis for sarcoma need to be quicker and more streamlined, but have neither been studied in detail in larger numbers before, nor in a direct comparison between two countries with different healthcare systems. Next to survival, health-related quality of life (HRQoL) and patient satisfaction can be influenced by experiencing delay. Currently, no data are available on how diagnostic delay impacts patient-reported outcomes among sarcoma patients.

Methods The QUEST is a longitudinal cohort study amongst adult sarcoma patients with a new sarcoma diagnosis in one of the participating study centers in The Netherlands or United Kingdom (enrolment began 01-02-2018, aiming to include 500 patients within 1.5 years). Patients will be invited before start of treatment and receive questionnaires on diagnostic pathway, risk factors and patient-

reported outcomes. Patient-reported outcome questionnaires will be completed again 3, 6, 12 and 24 months later. Data collection will be done within PROFILES, an international registry for cancer patient reported outcomes.

Clinical data will be collected from patients’ records.The primary endpoint is HRQoL at diagnosis. Descriptive statistics will be used to quantify diagnostic intervals. The association of diagnostic interval on HRQoL and survival will be explored in a number of ways to see if a cut off can be defined.

Expected outcomes These data will provide recommendations on what efforts to optimize diagnostic pathways are useful, both in terms of clinical outcomes and patient reported outcomes.

76. SSGXXII - Three versus five years of adjuvant imatinib as treatment of patients with operable GIST with a high risk of recurrence: A randomised phase III multicentre study by the Scandinavian Sarcoma Group

V Ramesh Bulusu, Cambridge University Hospitals NHS Foundation Trust Background: Three years of adjuvant imatinib is standard in patients with high-risk resected GIST. Yet many patients remain at high risk of recurrence after completing 3 years of adjuvant imatinib and may benefit from further adjuvant imatinib.

SSGXVIII study highlighted high mitotic index and non-gastric site as the most important predictive factors for recurrence.

Hypothesis: Further 2 years of adjuvant imatinib may improve recurrence-free survival (RFS) of patients who are at high risk of GIST recurrence despite completing 3 years of adjuvant imatinib.

Methods: Trial Design Open-label, 2 arm, prospective, randomised, multicentre, phase III trial.

300 patients with resected high risk GIST completing 3 years of adjuvant imatinib and

Page 83

Abstracts - Posters

associated with poor balance/gait, which link in with higher disability important in guiding rehabilitation strategies.

77. MS-275 inhibits sarcoma stress adaptation and enhances the effects of Doxorubicin and Cisplatin

Amal M EL-Naggar, Dept of Pathology & Laboratory Medicine, University of British Columbia; Dept of Molecular Oncology, BC Cancer Research Centre; Syam Prakash Somasekharan, Vancouver Prostate Centre, Vancouver, BC, V5Z 1M9, Canada; Xue Qi Wang, Dept of Molecular Oncology, BC Cancer Research Centre, Vancouver, BC, V5Z 1L3, Canada; Syam Prakash Somasekharan (3,8), Xue Qi Wang (2,8), Yemin Wang (2,8), Hongwei Cheng (3,8), Melvin Pan (2,8), Gian Luca Negri (2), Alberto Delaidelli (1,2), Bo Rafn (1,2), Jordan Cran (1,2), Fan Zhang (3), Haifeng Zhang (1,2), Nancy Kedersha (4), Anna Mandinova (5), Shane Colborne (6), Christopher S Hughes (2), Didier Surdez (7), Olivier Delattre (7), Yuzhuo Wang (3), David G Huntsman (1,2), Gregg B Morin (6), and Poul H Sorensen (1,2,9) 1. Dept of Pathology & Laboratory Medicine, University of British Columbia, Vancouver, BC, V6T 2B5, Canada 2. Dept of Molecular Oncology, BC Cancer Research Centre, Vancouver, BC, V5Z 1L3, Canada 3. Vancouver Prostate Centre, Vancouver, BC, V5Z 1M9, Canada 4. Brigham and Women’s Hospital, Harvard University, Boston, MA 02115, USA 5. Massachusetts General Hospital, Harvard University, Boston, MA 02114, USA 6. Michael Smith Genome Sciences Centre, Vancouver, BC, V5Z 4S6,

Canada 7. INSERM U830, Équipe Labellisée LNCC, PSL Université, SIREDO Oncology Centre, Institut Curie, Paris, France 8. Contributed equally to this work 9. Lead contact Sarcoma cells are continually exposed to acute stress within the tumor microenvironment, such as from reactive oxygen species (ROS) and toxic therapy. Successful adaptation to such stresses can then lead to emergence of clones with aggressive and/or metastatic capacity. We recently found that acute changes in mRNA translation forms a major component of stress-induced cell plasticity, through the induction of HIF1? (1) and G3BP1 translation

by YB-1, an RNA binding protein (2). G3BP1 then initiates stress granule (SG) formation, which are cytosolic aggregates containing stalled translation initiation complexes, G3BP1, YB-1, and silenced mRNAs. Genetically blocking either YB-1 or G3BP1 in sarcoma cells significantly inhibits SG formation in vitro under oxidative stress, and dramatically inhibits sarcoma metastasis in vivo, indicating that SG formation is critical for tumor invasion and metastasis. We now find that MS-275 (Entinostat) causes inhibitory acetylation of YB-1, blocking G3BP1 translation, and potently reducing SG formation, resulting in increased oxidative stress in sarcoma cells.

As Doxorubicin and Cisplatin are known to increase cellular ROS, we hypothesized that MS-275 may enhance the effects of such drugs. Indeed, survival plots of U2OS cells treated with Doxorubicin or Cisplatin, with or without MS-275, show that MS-275 sensitizes sarcoma cells to drugs that otherwise increase SG formation. Therefore, MS-275 inhibits sarcoma invasion and metastasis in vivo, and synergistically enhances the therapeutic effects of Doxorubicin and Cisplatin drugs in vitro. 1.

Cancer Cell, DOI: 10.1016/j.ccell.2015.04.003 2. Journal of Cell Biology, DOI: 10.1083/ jcb.201411047

Page 84

Abstracts - National Sarcoma Forum Your Voice Counts - Listening to Patients

Julie Woodford, London Sarcoma Service; Gemma French, London Sarcoma Service; Anne McTiernan, London Sarcoma Sewrvice The London Sarcoma Service (LSS) values state we will work in partnership with patients and families. This paper will report on two successful patient listening events held in October 2018. The LSS held two patient listening events at RNOH and UCLH. Patients and their family members were invited to participate and share their recent experiences of being treated within our service. A total of 19 people attended the events. Social media via the Trusts and Sarcoma charities was used to highlight the events and encourage attendance. Patients were also invited whilst they attended a clinic appointment or via postal invitation. The events were organised and facilitated by the LSS Sarcoma Improvement manager and the Lead Nurses. The Clinical Leads welcomed the participants and provided an overview of UK Sarcoma Services. Representatives from the LSESN SAG, Sarcoma UK and SCAT were also in attendance. The findings of the National Sarcoma Survey results were presented.

Listening to patients was very powerful. Many gave positive feedback and were grateful for receiving care and treatment in an expert centre. Some areas for improvement were also identified. Attending the event was reported as a particularly positive experience by all those who were present. They enjoyed meeting other patients who had had a sarcoma and whom had shared a similar experience. Many of those who attended planned to keep in contact after the event. All of those who attended wished to be invited to any future listening events.

Review of physiotherapy provision at Bone and Soft Tissue Sarcoma Centres across England

Lynsey Green, Nottingham University Hospitals NHS Trust; Debbie Artis, Leeds Teaching

Hospitals NHS Trust; Geraint Davies, The Robert Jones and Agnes Hunt Orthopaedic Hospital NHS Foundation Trust; Lucy Dean, The Royal Marsden NHS Foundation Trust; Abigail McCarthy, Royal National Orthopaedic NHS Trust; Natalie Morgan, Royal National Orthopaedic NHS Trust Aim: To assess variability in physiotherapy provision across specialist bone and soft tissue centres in England Method: Specialist bone and soft tissue sarcoma centres in England were invited to complete an online questionnaire. The survey asked 10 questions regarding the provision of physiotherapy services for sarcoma patients.

Results: Questionnaires were sent to the ten Soft Tissue Sarcoma Centres and the five Bone and Soft Tissue Centres in England. Twelve responses were received (7 soft tissue centres and 5 bone and soft tissue centres). Seven centres have a designated sarcoma physiotherapist who is an extended member of the MDT. Designated paediatric sarcoma physiotherapists are in post at 3 centres. Sarcoma patients at centres which do not have a specialised sarcoma physiotherapist are seen by physiotherapists linked to other specialities including burns and plastics, trauma and elective orthopaedics.

Patients are seen by physiotherapists at varying points in the pathway (diagnosis and pre-treatment n=8, inpatient post-treatment n= 10, post treatment surveillance clinic n=4, outpatient physiotherapy n= 8, specialist inpatient rehabilitation n=8, palliative and end of life care n=6). Only 25% of services were actively involved in physiotherapy research.

Conclusion: There is significant variability in physiotherapy provision for sarcoma patients across England.

We believe in order to receive high quality rehabilitation all patients should have access to

Page 85

Abstracts -National Sarcoma Forum

recurrence free are randomly allocated to Arm A or B in 1:1 ratio: Arm A: Further 24 months of adjuvant imatinib (total duration of adjuvant imatinib is 5 years) Arm B: Discontinue imatinib (total duration of adjuvant imatinib is 3 years) Follow up is for 10 years post-randomisation or until death.

Endpoints Primary: RFS after randomisation Secondary: Overall survival; GIST-specific survival; Adverse Events (CTCAE v3.0); Quality of Life Exploratory: Effect of tumour site on RFS; effect of tumour mutation type on RFS; effect of imatinib dose at randomisation on RFS; Tumour and blood molecular markers.

Study Progress Recruitment: Global=94 UK=17 Centres: 8 open UK accrual until March 2020.

Interactive workshop with the delegates of the NSF to identify the clinical utility of SAM

Julie Woodford, The Royal National Orthopaedic Hospital; Rachel Taylor, University College London Hospitals; Ana Martins, University College London Hospitals Background: There are currently no measures that will provide a holistic assessment specifically for patients with sarcoma. Although a recent symptom-based measure has been published1, this does not potentially assess patient’s emotional or social needs. The Sarcoma Assessment Measure (SAM) has been developed over the last two years as a sarcoma-specific outcome measure. The content is based on the analysis of 121 patients with a range of sarcoma-types aged 13 years onward to reflect the issues that have an impact on patient’s lives. Patient-reported outcome measures have the potential to be more than a research tool; they can be used in clinical practice to guide care delivery2.

The aims of this 30-60 minute workshop are to: 1. Define how measuring PRO would benefit patients with sarcoma 2. Explore how healthcare professionals could integrate the SAM into clinical practice 3. Explore organisational facilitators and barriers to integration into clinical practice Methods: Participatory methods using card visualisation and forcefield analysis will be used so delegates of the NSF can have an interactive discussion.

Summary: Through bringing together researchers and

clinicians we will identify ways in which current evidence will be able to improve the delivery of care to patients with sarcoma. Importantly, this will determine if the clinicians have the support they require to support patients; and if not, what they think will be of benefit, 1 Skalicky AM, Ghate SR, Perez JR, et al. Results of a Qualitative Study to Develop a Patient Reported Outcome Measure for Patients with 4 Subtypes of Soft Tissue Sarcoma.

Sarcoma 2017;2017:6868030. 2 Snyder CF, Aaronson NK, Choucair AK et al. Implementing patient-reported outcomes assessment in clinical practice: a review of the options and considerations. Quality of Life Research, doi: 10.1007/s11136-011-0054-x “The Clinical Nurse Specialist was really a stable point for me to go to for any sort of information or support” – the role of professionals’ expertise and support in patients’ experience

Ana Martins, University College London Hospitals; Rachel Taylor, University College London Hospitals; Julie Woodford, The Royal National Orthopaedic Hospital; Jeremy S Whelan, University College London Hospitals; Lindsey Bennister, University College London Hospitals; Lorna A Fern, National Cancer Research Institute; Craig Gerrand, The Royal National Orthopaedic Hospital; Maria Onasanya, University College London Hospitals; Lesley Storey, Queens University Belfast; Mary Wells, Imperial College Healthcare NHS Trust; Rachael Windsor, University College London Hospitals Background: Patients with sarcoma might attend multiple hospitals and sites for their treatment alongside with complex treatment regimes.

A survey on patients’ experiences shows that they benefit from clear navigation to help them understand what is happening and enhanced levels of reassurance are obtained by access to trusted quality information about their sarcoma1. Research shows that patients are more likely to understand their treatment plan, access services they need, cope with their illness and are better prepared

Page 86

for consultations and treatments if they have professional supporting care coordination2,3.

Aim: To explore the role professional’s expertise, provision of information and practical and emotional support on patient’s experiences.

Method: Participants across the UK were recruited by healthcare teams or through charities. Patients’ experience was shared in semi-structured interviews and focus groups, which were transcribed and analysed using Framework analysis.

Results: A total of 121 patients participated [49.6% male; 13-82 years old; with STS (62%), bone (28%) and GIST(10%)]. The expertise and experience of clinical teams restored patient’s trust in the healthcare profession, especially if their route to diagnosis had been long and complex. Patients valued their Clinical Nurse Specialist, described as having specialist expertise and being the key accessible professional for the multidisciplinary team.

Patients described the healthcare team working collaboratively to best support them. It was in the absence of clear communication and coordination that worse experiences were reported.

Conclusion: This study showed how professional’s expertise; information and emotional support played a key role in patients’ experience.

Physiotherapist Led Bone Tumour Surveillance Clinics

Geraint Davies, Robert Jones & Agnes Hunt Orthopaedic Hospital Background This new clinic has been developed in one of England’s five bone sarcoma services.

This service has seen increased need for new patient and surveillance clinic appointments without consultant expansion leading to above job plan clinics and

regular overbooking. Subsequently patients received short appointments with little holistic care. An innovative solution was required to manage this workload to give patients the time they deserve, a comprehensive assessment of their needs and to provide appropriate advice and support. A soft tissue sarcoma surveillance clinic had been commenced by the clinical nurse specialists which has proven to be safe and effective with high levels of patient satisfaction. Following the success of this the new bone tumour clinic was developed.

Bone tumour patients are diverse in terms of both age, ranging from adolescents to octogenarians, the surgery they have undergone and resultant disability. In addition to the usual social, economic, educational and ethnic diversity of the general population.

The aim was to provide bone tumour patients with their tumour and implant surveillance as well as an assessment of their overall physical and emotional well-being and provide treatment, advice and support all in a single hospital appointment with a Macmillan Physiotherapist.

Objectives • Reduce number of follow ups with consultant and increase their time with complex/new patients.

• Comprehensive physical and emotional assessment at each appointment.

• Chest and limb imaging is performed at each appointment as per standard protocol.

• Each appointment aims to promotes selfmanagement by increasing physical activity and wellbeing, managing the consequences of treatment and signposting to our support group, health and wellbeing events and information services • Frees up consultant appointments and provides cost saving • Part of the Recovery Package and Living with and beyond Cancer projects which is recognised by the NHS England five year forward view and cancer taskforce strategy.

Method • A selected group of patients with bone tumours are seen for follow up by a

Page 87

Abstracts -National Sarcoma Forum

a specialist sarcoma physiotherapist, regardless of geographical location. Barriers which prevent centres developing sarcoma physiotherapy services, such as funding limitations, need to be identified and addressed. A nationally agreed standard for physiotherapy provision may help to reduce inequalities in services for sarcoma patients.

Macmillan Physiotherapist rather than a consultant • This group demonstrate physical issues following surgery and their needs were not always being met even years after • All patients are known to the physiotherapist having been under their care as an inpatient giving continuity of care • Longer appointment times 30 minutes rather than 10 minutes • Consultant review remains available • Chest and limb imaging is performed at each appointment as per standard protocol.

All imaging is reported by Musculoskeletal Radiologists within 24 hours and results communicated back to the patient by the physiotherapist • All imaging reports are also signed off by the Orthopaedic Oncology Consultant

Annual Conference 2020 Glasgow SAVE THE DATE

Results • 119 patients were seen in the first 8 months of the service • Cohort increasing month on month • Releases capacity from consultant clinics leading to a more efficient patient centred service • Reduction in hospital visits for patients by combining surveillance, rehabilitation and advice in one appointment • Excellent feedback – 28 patients completed a feedback questionnaire - all 28 patients felt all their needs were met and were very satisfied with the information they received.

27 patients felt very satisfied with seeing a physiotherapist over a doctor, 1 satisfied.

• The service implementation has also proved a cost saving to the NHS of £13000.

Conclusion We believe this is the first service of its kind for bone tumour patients and we hope its success will provide a service model which can be adopted in other tumour units in the UK. The service is proving to be safe and effective.

26th & 27th February 2020 Hilton Glasgow, Scotland Page 88

Find out more at: www.britishsarcomagroup.org.uk

Note Paper

Note Paper

Page 90

Page 91

Note Paper

Note Paper

Page 92

Page 93

Note Paper

Programme & Abstract Book designed and produced by Echo Events & Association Management

Page 94

002_YON_UK_2018 Date of Prep: November 2018

Page 96