BSG Conference 2018 - Programme & Abstract Book

In partnership with:

Annual Conference 2018

Programme & Abstract Book Main Partner:

Tuesday 27th & Wednesday 28th February 2018 The Repertory Theatre, Birmingham

Contents Contents Welcome to BSG

3

General Info

4-5

BSG Update

5

About the BSG

6-7

Keynote Speakers

8-9

Main Conference Programme

10 - 17

NSF Programme

18

Posters

19 - 23

Sponsors

24

Exhibitor floorplan

25

Abstracts - Free Papers

27 - 44

Abstracts - Posters

45 - 86

Abstracts - NSF

87 - 88

BSG Conference 2019

89

Note Paper

90 - 94

Page 2

Welcome to the BSG Conference 2018 We would like to welcome you to Birmingham for this year’s British Sarcoma Group Meeting. For the first time, this year, the meeting is joint with the Irish Sarcoma Group.

The event has been running since 2004 and provides an opportunity for all health care professionals involved in the management of sarcoma to meet and share ideas. We were delighted this year to receive 115 abstracts which set a new record and were to be able to accept a large number of these, either as oral presentations or posters. In order to accommodate as much material as possible, the two day schedule is very busy.

We are also fortunate to be able to welcome a number of international visiting speakers including Fabio Efficace, Chairman of the EORTC quality of life group, Professor Brian O’Sullivan from the department of radiation oncology at the University of Toronto, Dr Sylvie Bonvalot, Chief Investigator of the STRASS trial and Dr Alessandro Gronchi, chairman of the EORTC sarcoma group.’

On day one we have an update on open clinical trials around the country followed by a session on quality of life chaired by Mr Roger Wilson. For those inspired by the first session on quality of life, there will be a further seminar that will be run in parallel later in the day. Alongside this there will be a presentation from our sponsors Pharmamar on uterine leiomyosarcomas.

We then move on to a session on basic

science and an update on the 100000 genome project in parallel with some free papers. In the afternoon there is a session on radiotherapy with an up-date on the STRASS trial, proton beam therapy and pre-operative radiotherapy.

In parallel to this there will be the National Sarcoma Forum. We end the day with a guest lecture from Professor Derek Alderson, President of the Royal College of Surgeons of England and Professor Paul Ridgeway, Consultant Surgeon at Tallaght Hospital, Dublin. The focus of this session will be the centralisation of services and promises to stir up some debate.

On day two we have an update on bone sarcomas in parallel with a sponsored symposium covering systemic treatment for metastatic soft tissue sarcomas from Eli-Lilly. After some free papers we have a pre-lunch time session covering radiology of soft tissue and bone sarcomas followed by an interactive quiz. In the afternoon the theme is of service organisation and delivery. First there is an overview of cancer services in Ireland. This is followed by a discussion about surgery, pathology, radiation and medical oncology. The final session will focus on issues important in the UK including commissioning, MDTs, follow up and data collection.

I hope you will agree that this is a varied and interesting schedule and I hope you will have the opportunity to see some of Birmingham and enjoy its vibrant nightlife.

Mr. Anant Desai

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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 Mezzanine on the 2nd floor.

EXHIBITORS & CHARITIES Sponsors & exhibitors will be located in the Mezzanine on the 2nd floor along with charity supporters.

CONFERENCE SESSIONS Will take place in the main conference room, The Studio. Parallel sessions take place in Suites 1 & 2.

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 Suite 3.

Access to the poster presentations will be available from Tuesday 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 27 - 88 THE CONFERENCE DINNER The BSG Conference Dinner will be held in The Studio on Tuesday 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 Wednesday 28th Feb. Awards will be presented for: • BSG Special Award • Poster Award 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.

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British Sarcoma Update WIFI Wifi is complimentary.

Depending on your location please log on to REP Guest WIFI or Library of Birmingham WIFI. It will ask for your email and then you will have full complimentary access.

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.

This year has been another significant one for the British Sarcoma Group.

We have improved our administrative processes and have put a more formal membership structure in place to represent better the views of the sarcoma community. This is essential as there is much work to do. We are challenged on many fronts – too many of our patients have conditions for which there is no cure, and some have conditions for which a treatment exists but is not funded. Many of us face extraordinary service pressures. Our hope is that BSG provides a forum to air these and other issues and exerts a positive influence where it is needed.

We very much hope that you enjoy the 2018 conference. Many thanks are owed to all of those who have worked so hard to bring it to you - these include Anant Desai and colleagues from Birmingham, Charles Gillham of the Irish Sarcoma Group, and Luan Suckling and the team at Echo Events. We are indeed fortunate to live within a collaborative and dynamic sarcoma community. Thanks to all of you for coming and contributing in so many different ways to what I am sure will be a memorable two days!

Craig Gerrand BSG President

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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.

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

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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, Newcastle Upon Tyne Hospitals Trust) • Ms Lindsey Bennister (Chief Executive, Sarcoma UK) • 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) • Ms Sarah Welby (Nurse Clinician, Christie Hospital, Manchester) • 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) 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

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Keynote Speakers Prof Derek Alderson Derek Alderson is an emeritus Professor of Surgery at the University of Birmingham. He was Professor of Surgery at the University of Bristol from 1997-2005 before becoming the Barling Professor of Surgery and Head of Department in Birmingham, a post he held until October 2015. A former President of the Association of Upper Gastrointestinal Surgeons of Great Britain and Ireland, his main clinical interest is in oesophago-gastric cancer surgery. He has a long association with the British Journal of Surgery. He was Editor in Chief from 2010 to 2016 and is now the Editor in Chief of BJS Open. In 2017 he was elected President of the Royal College of Surgeons of England.

Sylvie Bonvalot MD, PhD Sylvie Bonvalot received her MD with surgical specialty in 1990. Then, she completed her residency training in surgical oncology c/o Gustave Roussy (GR) in Villejuif, France. She was Chief of the sarcoma surgery at GR from 1997 to 2005 and joined Institut Curie in May 2015. Her clinical practice and research interests focused on sarcoma and desmoids. She graduated as PhD in 2006. She is the Principal Investigator of several French and international trials on sarcoma and desmoids and has authored more than 170 scientific publications. She serves as chairman of regional treatments of the French Sarcoma Group, is a member of the Sarcoma Task Force of the European Society for Medical Oncology (ESMO) and a member of EORTC Soft Tissue and Bone Sarcoma Group. She was admitted to membership in the European Surgical Association (ASA) in 2015. She served from 2005 to 2008 at the Board of Directors of the Connective Tissue Oncology Society (CTOS) and is a full Member of the National Academy of Surgery in France and professor of the Medical College of Paris.

Fabio Efficace, PhD Fabio is a member of the Scientific Board of the GIMEMA and a former Board member of European Organization for Research and Treatment of Cancer (EORTC) Quality of Life Group and of the Board of Directors of the “International Society for Quality of Life Research� (ISOQOL). He worked for several years at the EORTC in Brussels (Belgium) and has been Visiting Professor at the Memorial Sloan Kettering Cancer Center (MSKCC) in New York. He is also also the Chair Elect of the EORTC Quality of Life Group.

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Alessandro Gronchi, MD Alessandro Gronchi MD received his MD degree from Milan University (high honors) in 1992, and then completed his residency training in General Surgery at the San Raffaele General Hospital – Milan. During his residency he served for 2 years at the Italian Ministry of Foreign Affairs, working full time at the Lacor University Hospital in Gulu Uganda. He then completed his training with a fellowship in Surgical Oncology at the National Cancer Institute – Milan – Italy in 1998. He joined the faculty in the Department of Surgery at National Cancer Institute, as attending surgeon in the Sarcoma Service, in 1999. He visited the Memorial Sloan Kettering Cancer Center in New York in 2002. He has been chair of the Sarcoma Service at the National Cancer Institute – Milan – Italy since 2001

Brian O’Sullivan MD, FRCPC, FRCPI, FFRRCSI (Hon), FASTRO Brian O’Sullivan is a professor in the University of Toronto Department of Radiation Oncology and the Department of Otolaryngology / H&N Surgery. He holds the Bartley-Smith/Wharton Distinguished Chair in Radiation Oncology in the Department of Radiation Oncology at the Princess Margaret Hospital, University of Toronto. He has been a radiation oncologist in the Radiation Medicine Program at PMH since 1985. He received his medical degree at University College Dublin / National University of Ireland in 1976 and his specialist qualifications from the University of Toronto in 1983. Additional postgraduate training includes a fellowship in medical oncology, and a residency and clinical fellowship in radiation oncology, all at Princess Margaret Hospital in Toronto, Canada. He is a previous President of the Connective Oncology Society (CTOS) and former Head of both the Sarcoma and H&N Oncology Programs at PMH. He is now Director of the Princess Margaret Hospital – Shenzhen, China Sanming Radiation Oncology project. Professor O’Sullivan is the co-Chair of the US NCI Head and Neck Steering Committee, CTEP. He is the recipient of numerous international awards, and has published almost 380 peer-reviewed papers, 50 book chapters, and has written or edited 6 oncology textbooks. His interests include sarcoma and head and neck cancer, translational research, IMRT delivery and the principles of image guided radiotherapy. He is a member of the TNM Committee of the Union for International Cancer Control (UICC), Chair of the UICC Prognostic Factors Sub-Committee and represents the UICC as head and neck cancer and sarcoma liaison to the American Joint Committee on Cancer (AJCC). Since 2014 he has been a Commissioner of the ICRU.

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THE STUDIO

ROOM:

Bernadette Brennan - Update on the EuroEwings Trial Martin McCabe - REECur Trial Ramesh Bulusu, SSGXXII trial 3 versus 5 years of adjuvant Imatinib for GIST Meriel Jenney - An overarching study for children and adults with Frontline and Relapsed RhabdoMyoSarcoma Robin Jones, Phase I trials Sarah McDonald - The Sarcoma UK online clinical trials hub Bass Hassan - Results from the analysis of the LINES Phase II clinical trial in advanced Ewing sarcoma

THE STUDIO

08:50 - 09:02 09:02 - 09:14 09:14 - 09:26 09:26 - 09:38 09:38 - 09:45 09:45 - 09:50 09:50 - 10:00

ROOM:

10:00 - 11:15 Psychology/Quality of Life CHAIR: Roger Wilson 10:00 - 10:05 Introduction 10:05 - 10:17 Mr Matt Freeman (NHS England) - the Cancer Dashboard Metric 10:18 - 10:30 Dr Olga Husson (RMH/ICR) - Profiles and Sarcoma QofL at RMH 10:31 - 10:43 Dr Rachel Taylor (UCLH) - SAM - a Sarcoma specific QofL tool 10:45 - 11:00 GUEST LECTURER: Dr Fabio Efficace (EORTC) - The work of the EORTC QofL Group 11:01 - 11:15 Discussion - speakers plus Dr Derek Kyte

Update on open trials CHAIR: Mariam Jafri

08:50 - 10:00

ROOM: THE STUDIO

08:45 - 08:50 Welcome to BSG 2018

Anant Desai, British Sarcoma Group BSG Update Craig Gerrand, President, British Sarcoma Group

Registration

08:00 - 08:45

Programme - Day One: Tuesday 27th February 2018

Ian Tomlinson – Introduction into the 100K Genome

12:30 – 12:40

13:00 – 13:05 13:05 - 13:15

12:50 – 13:00

12:40 – 12:50

Project Adrienne Flanagan – Update on numbers submitted to GEL, planned & existing studies, GECIP model & Sarcoma Network for UK Andrew Beggs – Using clinically relevant pre-clinical models in cancer research Zoe Davison – BCRT Biobank update Discussion/Questions

THE STUDIO 100K Genome CHAIR: Anant Desai/Ian Judson

ROOM: 12:30 - 13:15

THE STUDIO Patient treatment of uterine Leiomyosarcoma and endometrial stromal sarcoma Prof Amant, Belgium Symposium sponsored by PharmaMar - Surgical management - Systemic management including chemotherapy and hormonal treatment - Patient derived xenograft as preclinical model - How to avoid morcellation of uterine sarcomas?

- mTor dual blockade: a promising tool for systemic treatment of uterine Leiomyosarcoma?

Morning Refreshments (Poster viewing in Suites 1&2)

ROOM: 11:45 - 12:30

11:15 - 11:45

Free Papers (parallel) CHAIR: Jonathan Gregory/ Sam Ford Dr Eugenie Younger - Health-related quality of

12:45 - 13:15 12:45 - 12:52

13:06 - 13:13

12:59 - 13:06

12:52 - 12:59

12:35 - 12:45

12:05 - 12:35

life in patients with advanced soft tissue sarcomas treated with chemotherapy: study protocol Elizabeth Roundhill - Profiling Ewing’s sarcoma patient-derived cancer stem-like cells to identify biomarkers of risk and potential targets for the design of novel treatment.

Tomohiro Fujiwara - Preoperative Surgical Risk Assessment in Osteosarcoma Based on the Proximity to the Major Vessels Daniel Tong - The role of blood parameters in predicting success of pre-operative radiotherapy in sarcoma patients

Quality of Life

CHAIR: Roger Wilson Introduction Dr Derek Kyte (CPROR - Birmingham University) What are PROs/PROMs and challenges using them Workshop/discussion with either: Dr Aisha Miah (RMH) - a PRO based study in Advanced Sarcoma, or Dr Rachel Taylor & Dr Olga Husson Challenges in developing sarcoma specific tools Discussion

11:45 - 12:45 11:45 - 11:47 11:47 - 12:05

SUITES 1 & 2

ROOM:

THE STUDIO

Radiotherapy CHAIR: Charles Gillham

GUEST LECTURER: Sylvie Bonvalot - STRASS - morbidity of pre-op radiotherapy

ROOM:

14:15 - 15:15

14:15 - 14:35

THE STUDIO

15:15 - 15:22 Robert Grimer - Prediction of recurrence after sarcoma treatment. Can it help plan follow up?

15:22 - 15:29 James Monteiro de Barros - A multi-centre, retrospective study of 408 retroperitoneal,

abdominal and pelvic schwannomas: Recommendations for management & surveillance 15:29 - 15:36 Robin L Jones - Efficacy of olaratumab, a human anti–platelet-derived growth factor α

(PDGFRα) monoclonal antibody, in combination with doxorubicin (Dox) vs Dox alone, in

advanced soft-tissue sarcoma (STS) – a subanalysis of the first line population in the

JGDG study 15:36 - 15:43 Ana Martins - A national collaboration to develop a sarcoma-specific patient report

outcome measure: Phase 1 - Exploring patient experiences 15:43 - 15:50 Fransiscus Kerans - Magnetic Nanoparticle Bearing Mesenchymal Stem Cells For

Magnetic Hyperthermia Treatment of Sarcomas 15:50 - 15:57 Michael Perry - Is Denosumab an Effective Treatment For Giant Cell

Tumor Of Bone in the Pelvis and Sacrum?

15:15 - 16:18 Free Papers CHAIR: Andrew Hayes / David Peake

ROOM:

14:35 - 14:45 Cath Coyle - Protons: Who, why, what, where 14:45 - 15:05 GUEST LECTURER: Brian O’Sullivan - Do we think we know everything about pre-operative radiotherapy 15:05 - 15:15 Questions

Lunch (Poster viewing in Suites 1&2)

13:15 - 14:15

Programme - Day One (Continued)

Sponsored by:

See page 18 for the NSF programme

14:15 - 16:15 NATIONAL SARCOMA FORUM

ROOM: SUITES 1 & 2

Afternoon Refreshments (Poster viewing in Suites 1&2)

16:18 - 16:45

GUEST LECTURER: Derek Alderson – Specialisation and centralisation of complex surgery in the NHS GUEST LECTURER: Paul Ridgway – Sarcoma centralisation in Ireland: Much done, more needed Discussion/Question

Conference Close

Drinks Reception - taking place in The Foyer at the REP Theatre BSG Conference Dinner - taking place in The Studio at the REP Theatre

16:45 – 17:00 17:00 – 17:15 17:15 – 17:30

17:30

19:30 20:00

ROOM: THE STUDIO 16:45 - 17:30 BSG Guest Lecture: Structuring Specialist Services CHAIR: Charles Gillham/Deirdre O’Mahony

Jonathan Bray - Soft tissue sarcoma in dogs: what can we learn from man’s best friend?

Sarah Kalus - Bone scintigraphy vs whole body MRI in the staging of Ewing sarcoma of bone: a 10-year review Magdalene Joseph - The development of a Hepatitis B (HBV) screening programme for patients with Gastrointestinal Stromal Tumours (GIST) on Imatinib treatment - A Local Experience at Guy’s and St Thomas’ NHS Foundation Trust (GSTT)

15:57 - 16:04 16:04 - 16:11

16:11 - 16:18

THE STUDIO

Welcome to day two Charles Gillham, Irish Sarcoma Group

ROOM:

08:45 - 08:50

Sandra Strauss - Osteosarcoma update Craig Gerrand, Jeremy Whelan - Ewings Update Catherine Coyle - Chordoma Update Lee Jeys/Michael Parry - Chondrosarcoma Update

THE STUDIO

08:50 – 09:05 09:05 – 09:30 09:30 – 09:45 09:45 – 10:00

ROOM:

10:00 - 10:07

10:07 - 10:14 10:14 - 10:21 10:21 - 10:28 10:28 - 10:35

10:35 - 10:42 10:42 - 10:49

09:00 - 09:30

NHS Foundation Trust

Medical Oncologist, University Hospitals Bristol

Dr Adam Dangoor MD MRCP, Consultant

Selecting systemic treatment for mSTS Symposium sponsored by Lilly Oncology

Kwok Wong - Incidence and survival of bone and soft tissue sarcoma (STS) in England between 1996 and 2015: An analysis from the National Cancer Registration and Analysis Service Gargi Kothari - Treatment and outcomes of borderline and malignant Phyllodes tumours of the breast Hannah Tattersall - Diagnostic Accuracy of Percutaneous Biopsy in Retroperitoneal Sarcoma Dr Nilesh Gurjar - MRI Differentiation of Low-grade from High-grade Pelvic Chondrosarcoma Dr Scheryll Alken - Survival of paediatric and adolescent / young adult (AYA) cancer patients with sarcoma in Ireland during 1994- 2014: comparisons by age Robert Grimer - The ITAP project – successful or not?

Orlaidh Burke - Centralising Sarcoma Services in Ireland

10:00 - 10:45 Free Papers CHAIR: Ian Judson / Myles Smith

Bone Sarcoma Update CHAIR: Jonathan Gregory

08:50 - 10:00

ROOM: SUITES 1 & 2 ROOM: THE STUDIO

Registration

08:00 - 08:45

Programme - Day Two: Wednesday 28th February 2018

Nicholas Eastley - Circulating nucleic acid characteristics of non-metastatic STS patients Sandra J Strauss - A Phase I Study of the Poly-ADP Ribose Polymerase (PARP) inhibitor, Niraparib (NIR) in combination with Temozolomide (TMZ) in patients with advanced incurable Ewing Sarcoma (ES): Final results of SARC025 Arm 1 Dr Lalit Pallan - Management of Gynaecological Sarcomas (GS) at Pan Birmingham Gynaecological Cancer Centre (PBGCC)- A 10

12:00 - 12:07 12:07 - 12:14

ROOM: 12:30 - 12:45

12:21 - 12:28

12:14 - 12:21

THE STUDIO Free Papers CHAIR: David Gourevitch / Mariam Jafri

ROOM: 12:00 - 12:30

THE STUDIO Sarcoma Awareness Project 2017 Coonoor Chandrasekar, Roger Wilson and Craig Gerrand

year experience Kwok Wong - A National Cancer Registration and Analysis Service (NCRAS) Systemic Anti-Cancer Therapy (SACT) analysis to investigate the use of Mifamurtide - Muramyl Tripeptide Phosphatidylethanolamine (MTP-PE) in patients with non-metastatic, resectable osteosarcoma

Start up meeting for the BSG systemic therapies advisory group. Representatives from all networks welcomed.

SACT Start-up Meeting

Hassan Douis - Imaging of soft tissue tumours Asif Saifuddin - Imaging of malignant bone tumours Interactive MDT Quiz

SUITES 1 & 2

11:15 - 11:30 11:30 - 11:45 11:45 - 12:00

11:15 - 12:00

Radiology CHAIR: Alex Gronchi

ROOM:

THE STUDIO

11:15 - 12:00

Morning Refreshments (Poster viewing in Suites 1&2)

ROOM:

10:49 - 11:15

THE STUDIO

The Irish Sarcoma Group CHAIR: Charles Gillham / Anant Desai

Dr Jerome Coffey - An overview of cancer services in Ireland Prof Gary O’Toole - Surgical Services for Sarcoma in Ireland Prof Aurelie Fabre - Pathological Evaluation for Sarcoma Service in Ireland Dr Deirdre O’Mahony - Medical Oncology Service and the Irish Sarcoma Group Dr Charles Gillham - Radiation Oncology Service and the Irish Sarcoma Group Discussion

THE STUDIO

ROOM:

13:45 -14:30

1345 – 1352 1352 – 1359 1359 – 1406 1406 – 1413 1413 – 1423 1423 – 1430

ROOM:

Jayshree Veeratterapillay and Anthony Waton - Tomotherapy based IMRT in the treatment of soft tissue sarcomas (STS), Northern Centre for Cancer Care (NCCC), 8 years experience Shailen Patel - A systematic review of population-based studies examining outcomes in primary retroperitoneal sarcoma surgery Samim Ghorbanian - Does the sarcoma MDT need transformation?

14:37 - 14:44

14:44 - 14:51 14:51 - 14:58

ROOM: THE STUDIO 15:00 - 16:15 Service Organisation CHAIR: Craig Gerrand 15:00 – 15:15 Jeremy Whelan - Can we commission better services for patients with sarcoma?

15:15 – 15:35 Sandra Strauss – Can national data help services develop?

Lucinda Gosling - Identification of adult knee primary bone tumour symptom presentation: A qualitative study

14:30 - 14:37

14:30 - 15:00 Free Papers CHAIR: Adam Dangoor/Mike Parry

Lunch (Poster viewing in Suites 1&2)

12:45 - 13:45

Programme - Day Two (Continued)

Sam Hackett – Patient centred follow-up Craig Gerrand – Sarcoma MDTs – survey results and next steps Questions

16:30

Conference Close

ROOM: THE STUDIO 16:15 - 16:30 Closing Remarks

Anant Desai/Craig Gerrand Award presentations

15:35 – 15:50 15:50 – 16:05 16:05 – 16:15

National Sarcoma Forum: Programme ROOM 14:15 - 14:25

SUITES 1 & 2 Welcome Andrea Slade/Fiona Fitzgibbons

14:25 - 14:55

14:45 - 14:55

Person Centred Care & the Recovery Package

GUEST LECTURER: Dr Natalie Doyle, Consultant Nurse Discussion/Questions

14:55 - 15:45 14:55 - 15:05 15:05 - 15:15

15:15 - 15:25

15:25 - 15:35

15:35 - 15:45

FREE PAPERS Caroline Pemberton - Implementation of Nurse led Clinics Una Hayden - Nurse Specialist Support in Sarcoma services: an analysis in unscheduled workflow Debbie Artis - Measuring the impact of Outpatient Physiotherapy intervention with a patient reported outcome tool, MYMOP Helen Stradling - Sarcoma UK support line update Questions

15:45 - 16:00 15:55 - 16:00

Catherine Coyle – Preparing patients for Protons in Manchester Questions

16:00 - 16:15

Closing discussion and close

16:15

Afternoon Refreshments

National Sarcoma Forum Sponsor:

Thank you to our Charity supporters:

Page 18

Posters Dr. Bindu George, Nottingham University Hospitals NHS Trust

1. Factors influencing people’s ability to maintain their activity levels during chemotherapy for soft tissue sarcoma - a qualitative study Sarah Dewhurst, Royal Marsden Hospital

9. Preclinical Testing Of TRAIL Therapeutics For Sarcoma Zakareya Gamie, Newcastle University

2. Indeterminate Superficial Soft Tissue Tumours - Relationship of Lesion Depth to Differential Diagnosis Michael Khoo, Royal National Orthopaedic Hospital

3. Secondary Amputations For Soft Tissue Sarcoma: Recurrent Tumour Or Treatment Effect Lynsey Green, East Midlands Sarcoma Service, Nottingham

4. Palliative radiotherapy in elderly patients with cutaneous angiosarcoma of head and neck Cristiana Alzamora, University Hospital Southampton NHS Foundation Trust

5. Validation of a novel NGS approach to improve sarcoma diagnosis using FFPE Lauren McConnell, Centre for Cancer Research and Cell Biology

6. Heat Shock Protein (HSP27, HSP70 and HSP90) expression in Myxofibrosarcoma of the extremities –predicts prognosis?

Ayman Al Amri, Royal Liverpool and Broadgreen University Hospital

7. Why do we need SAM”? A narrative review of the impact of sarcoma on psychosocial wellbeing Lesley Storey, Queen’s Univerisyt Belfast

8. A rare case of primary intimal sarcoma of the pulmonary artery

10. Radiotherapy for the palliation of advanced sarcomas - The effectiveness of radiotherapy in providing symptomatic improvement for advanced sarcomas in a single-centre cohort.

Hannah Tween, Queen Elizabeth Hospital Birmingam

11. Transforming Sarcoma Services – the role of a Sarcoma Service Improvement Manager. Everyone needs one?

Gemma French, University College London Hospital

12. 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.

Victoria Ingleson, Cambridge University Hospitals NHS Foundation Trust

13. Pazopanib in Advanced Soft Tissue Sarcoma; Scottish Study Update Sara Walker Beatson, West Of Scotland Cancer Centre

14. Quantitative analysis of complications following sarcoma surgery with or without radiotherapy. East Midlands Sarcoma Service Experience Moataz Abdelreheem, East Midlands Sarcoma Service, Nottingham

Page 19

15. Uterine Leiomyosarcoma. Referral patterns to the Sarcoma Unit at The Royal Marsden Hospital Edward Nganga, Royal Marsden Hospital

23. Radiology recording of sarcoma MDT outcomes T Bean, Plymouth Hospitals NHS Trust

16. Secondary angiosarcoma in previously irradiated breast cancer patients - review of a regional experience Elena Cojocaru, Cambridge University Hospital, Oncology Department

24. Primary abdominal and retroperitoneal sarcomas: changing outcomes over time at a single institution Max Almond, University Hospital Birmingham

25. The Role of DMSA Imaging in Surgical Planning for Patients with Retroperitoneal Soft Tissue Sarcoma Miss HL Tattersall College of Medical and Dental Sciences, University of Birmingham

17. Prolonged activity of Trabectedin in Myxoid Liposarcoma – A Case Report Dr Radha Todd, Northern Centre for Cancer Care, Newcastle

26. Is thalidomide of use in angiosarcoma - what we can learn from dogs.

Jonathan Bray, Fitzpatrick Referrals Oncology and Soft Tissue

18. Claudin-3 localization in Schwann cell - like- cell and Endothelial cell in Neurofibromas M.H.Sulaiman Ahmadu Bello, University Zaria, Nigeria

27. Retroperitoneal sarcomas; MDT burden and diagnostic service Olivier Dick, NUH

19. Fibromatosis of the breast – A 10 year institutional experience and review of the literature Michael R Boland, Dept. of Breast Surgery, St James’ Hospital, Dublin 8, Ireland

28. Mechanisms Of Failure and Long Term Survival Of Total Femur Replacements.

Manuel Ricardo Medellin Rincon, Royal Orthopaedic Hospital

20. A Mother’s Grief: New Insights into Osteosarcoma from the Application of Aerospace Engineering Principles to PatientDerived Genomic and Medical History Data Theresa Beech, NASA

21. Restructuring the Sarcoma MDT: our experience as a tertiary referral centre Jennifer Murphy, Royal Orthopaedic Hospital, Birmingham

22. Neutrophil:Lymphocyte Ratio, Albumin and Platelets In Early Relapse In Soft Tissue Sarcoma Robert U Ashford, East Midlands Sarcoma Service

29. Lengthening Total Femur Arthroplasty, Comparison Between Non-Invasive And Minimally Invasive Prosthesis Manuel Ricardo Medellin Rincon Royal Orthopaedic Hospital

30. Prognostic Factors In The Management Of Giant Cell Tumours Of Extremity Bones With Pathological Fracture Manuel Ricardo Medellin Rincon, Royal Orthopaedic Hospital

31. Diagnostic accuracy of image-guided needle biopsy for Ewing sarcoma of bone: a 10-year review Sarah Kalus, Royal National Orthopaedic

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32. Desmoid Fibromatosis Secondary To ACL Reconstruction With An Endobutton Nicholas Eastley, East Midlands Sarcoma Service

41. The effects of metabolic modulation and immunogenicity in Osteosarcoma Kheshwant Gill, Cancer Research at University College Cork

33. Ten Years Egyptian Experience In Treating Bone Sarcomas Ehab Saad, Department of clinical oncology, Cairo University, Egypt

42. Mapping the Site of Local Recurrence in Retroperitoneal Liposarcoma (RPLPS) Sandro Pasquali, Istituto Nazionale dei Tumori, Milan

34. Single Centre Outcomes from Retroperitoneal Sarcoma Surgery James Bunce, Nottingham University Hospitals NHS Trust

43. Anthracycline-related cardiotoxicity in Ewing’s and Osteosarcoma patients N Osifodunrin, Royal Hospital for Children, Glasgow

35. Evaluating the Cost of a Retroperitoneal Sarcoma Service at a Single Centre James Bunce, Nottingham University Hospitals NHS Trust

44. A Prognostic Hypoxia Related Gene Signature for Soft Tissue Sarcoma Laura Forker, University of Manchester

45. Extra-axial chordoma of the anterior chest wall Ben Davies, Royal National Orthopaedic Hospital

36. Is radiation underutilised for palliation in paediatric osteosarcoma?

Neil Wallace, Radiotherapy Department, Cork University Hospital

46. A Proof-of-Concept Study using DCEMRI and Serum Proteomics to Investigate the in vivo Mechanism of Action (MoA) of trabectedin in Advanced Uterine Leiomyosarcoma (uLMS) and Non-Uterine Leiomyosarcoma or Liposarcoma within the Abdomen / Pelvis Dr Robin Jones, Royal Marsden Hospital

37. Long term use of Trabectadin – 3 cases showing efficacy and tolerability Dr Lesley Speed, University Hospitals of Leicester NHS Trust

38. Dąbska Tumour --- A Rare Case Report in Adult and Literature Review Qi Yin, Royal Liverpool University Hospital

39. The value of a telephone virtual clinic for a regional sarcoma service Anita Pabla, East Midlands Sarcoma Service

40. Diagnostic accuracy of CT-guided percutaneous bone biopsy in a tertiary musculoskeletal oncology centre.

Pedone Luigi, The Royal National Orthopaedic

47. A pilot study of patient reported outcomes when treated with Trabectedin for unresectable and/or metastatic soft tissue sarcoma Dan Stark, Leeds Teaching Hospitals NHS Trust Leeds Institute of Cancer and Pathology, St. James’s University Hospital

48. Radiation Induced Angiosarcoma of the Breast – A Growing Challenge in the Era of

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Breast Conserving Surgery Philippa Jackson, Leeds Teaching Hospitals

49. Knowledge and attitudes towards Adolescent and Young Adult (AYA) Oncology amongst cancer care providers in Ireland Scheryll Alken, St James’s Hospital

50. Staging rationale in soft tissue sarcoma Richard Hutchinson, Newcastle Hospitals NHS Foundation Trust

51. Quality of life and reintegration into normal life after extremity soft tissue sarcoma surgery Srikanth T, Green L, Wilshaw N, Steele KH, Tamimy MS, Raurell A, Ashford RU East Midlands Sarcoma Service, Nottingham

57. Concurrent venous thromboembolism and lower limb soft tissue sarcoma – Problem and algorithm of management Vinay Itte

58. Rate and Patterns of Local Recurrence Following Radical Treatment of Soft Tissue Sarcoma Rebecca Shakir, Oxford University Hospitals NHS Foundation Trust

59. MRI characteristics of Atypical Lipomatous Tumours Onkarpreet Jassel, UoN

52. Abdominal wall reconstruction after Desmoid tumour resection- surgical technique and management – A single centre case series Vinay Itte, Leeds Teaching Hospital NHS Trust

53. Resection of Chest Wall Chondrosarcoma and Reconstruction with 3-D Printed Titanium Implant Ira Goldsmith, Morriston Hospital, Swansea, SA6 6NL, Wales

54. PNSTs: our experience Dr Iain Macleod, Nottingham University Hospitals NHS Trust

55. The cost of centralising sarcoma services for patients, a single centre experience.

Karen O’Meara, Royal Marsden NHS Foundation Trust

56. Outcomes of patients receiving Irinotecan and Temozolamide for relapsed/ refractory Ewing’s sarcoma (ES) Helen Clarke, Queen Elizabeth Hospital

60. Is whole bone MRI safe for planning resection or limb salvage surgery in long bone osteosarcoma and Ewing Sarcoma?

Mr James Barnett, Royal National Orthopaedic Hospital, Stanmore

61. Wild type GastroIntestinal Stromal Tumours: an adult pooled analysis compared with paediatric controls Cillian Clancy, Dept Surgery, Tallaght Hospital

62. Demographics and outcomes of extracranial chordomas and chondrosarcomas treated with high dose external beam photon radiotherapy: The UCLH centre experience.

Dr Pei LIM, UCLH

63. Working together to ensure sarcoma patients receive the high-quality information they deserve.

Hannah Birkett, Bone Cancer Research Trust

64. Ambulation outcomes following lower limb amputation Geraint Davies, Robert Jones & Agnes Hunt Orthopaedic Hospital NHS Foundation Trust

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65. Regorafenib in the third line setting for gastrointestinal stromal tumours (GIST): A single centre experience Lucy McAvan, University Hospitals Birmingham NHS Foundation Trust

73. 5 year experience of sarcoma at UHL S Jennings, Department of Surgery, University Hospital Limerick, Limerick, Ireland

66. Should we be offering pre-op radiotherapy to our sarcoma patients?

Timothy Mitchell, Beatson West of Scotland Cancer Center

67. Multifocal Dermatofibrosarcoma Protuberans --- A Rare Case Report Qi Yin, Royal Liverpool University Hospital

68. Surgical excision margins in soft tissue tumours – An audit of 91 patients Christian Gray Stephens, Royal National Orthpaedic Hospital

69. Characteristics of patients diagnosed with angiosarcoma presenting to a Sarcoma Centre Gurvin Chander, Universirty Hospital Birmingham

70. The use of mifamurtide in completely resected high grade osteosarcoma in two University Hospitals.

Haider Abbas, University Hospital Birmiingham NHS Foundation Trust

71. Imaging appearances of gastrointestinal stromal tumours (GISTs)-Is there a correlation with precision computer tomography (CT) findings, genomics and outcomes?

Dr Balraj Dhesi, University Hospitals Birmingham NHS Foundation Trust

72. Review of Deep Infections Associated with Endoprosthetic Replacement Ian Crowther, Newcastle upon Tyne Hospitals NHS Foundation Trust

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Thank you to our Sponsors Main Partner:

PharmaMar are proud to be the main sponsor again of the BSG 2018. The highly regarded Prof Amant from the Netherlands Cancer institute “Antoni Van Leeuwenhoek” will be talking on “The treatment of Uterine Leiomyosarcomas”.

Plus Dr Radha Todd the experienced Sarcoma Specialist from the Royal Victoria Infirmary, Newcastle, will present a case study on “The Long term usage of Trabectedin” www.pharmamar.co.uk

Premium Sponsor:

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

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. www.takeda.co.uk

Thank you to our Exhibitors:

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6. Sarcoma UK

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7. Takeda Oncology

Baby Change Facility

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2. GIST Support UK

HDRY

PLANT ROOM

In partnership with:

Exhibitors

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2018

27th & 28th February 2018, Birmingham

8. Lilly Oncology

CATERING POINT

9. PharmaMar

Annual Conference

Exhibitor Floorplan

3. Zimmer Biomet UK

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.

Please refer to the Summary of Product Characteristics for details on the full side-effect profile and drug interactions of Mepact. Adverse events should be reported. Reporting forms and information can be found at https://yellowcard.mhra.gov.uk/. 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. Š Takeda UK Limited, 2017. 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

Date of preparation: January 2017

DAY ONE - SESSION: 12:45 - 13:15 Health-related quality of life in patients with advanced soft tissue sarcomas treated with chemotherapy: study protocol

questionnaire, treatment expectations and decision regret scale. Ethical approval; 17/ NI/0197.

Dr Eugenie Younger, The Royal Marsden Hospital NHS Foundation Trust; Professor Winette van der Graaf, The Royal Marsden Hospital NHS Foundation Trust and The Institute of Cancer Research, London UK; Dr Olga Husson, The Institute of Cancer Research, London, UK; Dr Robin Young, Weston Park Hospital, Sheffield, UK; Dr Michael Leahy, The Christie NHS Foundation Trust, Manchester, UK; Dr Ingrid Desar, Radboud University Medical Centre , Nijmegen, The Netherlands; Dr Robin Jones, The Royal Marsden NHS Foundation Trust and The Institute of Cancer Research.

Background Chemotherapy for the majority of patients with advanced soft tissue sarcomas (STS) is given with palliative intent. Health-related quality of life (HRQoL) in these patients is not well-described.

Methods This is a multicentre (n=3), longitudinal cohort study. One hundred and thirtytwo patients (aged ≥18 years) with advanced STS, eligible to receive 1stline chemotherapy, will be recruited over 18 months and followed up for (maximum) two years. A cohort of patients starting 3rdline chemotherapy will also participate. After informed consent, patients will complete online electronic questionnaires using the PROFILES data management system, or paper versions if preferred. Questionnaires will be completed at baseline, Day one of each cycle, at the end of chemotherapy and three-monthly during follow-up.

Validated questionnaires will be used; EORTC QLQ-C30 and financial toxicity, workability index, decisional control preferences, decision conflict scale, quality-quantity

Statistics Change in global HRQoL will be calculated using a paired sample t-test from baseline to four cycles (last observation carried forward). Change in HRQoL functioning scales and symptoms will be analysed using mixed models. Association between sociodemographic, clinical factors, radiological response and financial toxicity with HRQoL will be analysed using multivariate mixed models. Patient preferences, expectations, decisional conflict and regret will be described using mean or median as appropriate.

Expected outcomes These data will enable future patients with advanced STS to make a more well-informed decision about chemotherapy, and provide a benchmark for future studies evaluating the efficacy of novel systemic therapies in STS. DAY ONE - SESSION: 12:45 - 13:15 Profiling Ewing’s sarcoma patientderived cancer stem-like cells to identify biomarkers of risk and potential targets for the design of novel treatment

Elizabeth Roundhill, Children’s Cancer Research Group, Leeds Institute of Cancer and Pathology, University of Leeds; Susan Burchill, Children’s Cancer Research Group, Leeds Institute of Cancer and Pathology, University of Leeds; Elizabeth Roundhill(1), Mariona Chicon-Bosch(1), Alastair Droop(2), Kenneth Rankin(3), Dan Yeomanson(4), Michael Parry(5), Lee Jeys(5), Susan Burchill(1) 1 Children’s Cancer Research Group, Leeds Institute of Cancer and Pathology, St. James’s University Hospital, Leeds UK. 2 MRC Medical Bioinformatics Centre, University of Leeds, Leeds, UK.

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Abstracts - Free Papers

3 Freeman Hospital, Newcastle Upon Tyne, UK. 4 Sheffield Children’s Hospital, Sheffield UK. 5 The Royal Orthopaedic Hospital, Birmingham, UK.

Conclusions We have identified 2 targets that may be used to identify and eradicate ES-CSCs

Background Cancer stem-like cells (CSCs) are capable of self-renewal and may be responsible for disease progression and relapse (Schatton et al. Bioessays, 2009.31:p1038-49). Therefore, eradication of Ewing’s sarcoma (ES) CSCs is expected to improve outcome for patients.

In this study we have characterised paired ES and ES-CSCs to identify potential prognostic biomarkers of risk and targets for treatment.

Methods ES-CSCs were isolated using a functional self-renewing assay from diagnosis tumour biopsies following patient consent (GenoEwing, IRAS-167880). CD99 protein expression was determined by immunocytochemistry, western blot and flow cytometry. The EWSR1 translocation was confirmed by FISH using the Vysis EWSR1 Break apart Probe (Abbott Laboratories) and RT-PCR. Total RNA libraries were prepared and sequenced using the HiSeq3000 (illumina®). Reads were pre-processed using fqtools and aligned by STAR. Differential expression was determined using DESeq. Genes were ranked on adjusted p value and log2 fold change; candidates were validated by RTqPCR Findings ES cells were successfully isolated and propagated from 47% (8/17) of tumours; all expressed CD99 (24/24) and contained the EWSR1-Fli1/ERG translocation (17/17). Novel EWSR1 translocations were sequenced in 7 cases. The RNA profile of parental cultures and daughter ES-CSCs was compared. Two genes were taken forward for validation with adjusted p values of 1.45x10-15 and 5.74x10-14, respectively. Increased expression was confirmed in the ES-CSCs compared to corresponding parental cells

by RTqPCR (p<0.0001).

Funded by ESRT, BCRT, UoL. 1. Schatton, T., N.Y. Frank, and M.H. Frank, Identification and targeting of cancer stem cells. Bioessays, 2009. 31(10): p. 1038-49.

2. Droop, A.P., fqtools: an efficient software suite for modern FASTQ file manipulation.

Bioinformatics, 2016. 32(12): p. 1883-4.

3. Dobin, A., et al., STAR: ultrafast universal RNA-seq aligner. Bioinformatics, 2013. 29(1): p. 15-21.

4. Anders, S. and W. Huber, Differential expression analysis for sequence count data.

Genome Biol, 2010. 11(10): p. R106.

DAY ONE - SESSION: 12:45 - 13:15 Preoperative Surgical Risk Assessment in Osteosarcoma Based on the Proximity to the Major Vessels

Tomohiro Fujiwara, The Royal Orthopaedic Hospital; Adesegn Abudu, The Royal Orthopaedic Hospital; Tomohiro Fujiwara - Fellow in Orthopaedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K; Manuel R Medellin – Fellow in Orthopaedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K; Evans Scott - Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K; Jonathan Stevenson - Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital Birmingham, U.K; Michael Parry - Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K; Jonathan Gregory Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K; Roger Tillman - Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K; Lee Jeys Professor - Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham,

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DAY ONE - SESSION: 12:45 - 13:15 The role of blood parameters in predicting success of pre-operative radiotherapy in sarcoma patients

Introduction: Although the proximity to the major vessels is a definitive factor that determines the surgical strategy in osteosarcoma, the risk of surgical treatment according to the proximity has never been investigated.

The aim of this study was to clarify the risk of recurrence or survival based on the proximity to the major vessels.

Daniel Tong, Royal Marsden Hospital; Aisha Miah, Royal Marsden Hospital; Shane Zaidi, Dirk Strauss, Andrew Hayes, Myles Smith, Nicos Memos - Royal Marsden Hospital

Patients and Methods: A total of 230 patients with high-grade non-metastatic osteosarcomas in extremities were included into the study. Tumor-related variables included the vascular proximity on preoperative MRI after neoadjuvant chemotherapy, which were categorized into four types: type 1, > 5 mm; type 2, â&#x2030;¤ 5 mm, > 0 mm; type 3, attached; type 4, surrounded.

Results: The overall incidence of local recurrence (LR) was 8% and the 5-year overall survival (OS) was 69%. Limb salvage rate based on the proximity type was 91%, 87%, 50%, and 0%, respectively. LR incidence was 6%, 7%, 12%, and 0%, and 5-year OS was 84%, 79%, 51%, and 67%, respectively. In type 3, LR-free survival by limb salvage were significantly poorer than amputation/rotationplasty, while the latter offered no survival benefit.

However, the risk of LR by limb salvage did not significantly differ from amputation/ rotationplasty when the patients were good responders or the vascular attachment were less than half circumference or 10 mm longitudinally.

Conclusions: Even if the tumors were attached to major vessels on preoperative MRI, the risk of limb salvage was minimal if the patients were good responders or the vascular attachment was limited.

Background: Radiotherapy is recommended in high grade sarcoma, deep seated large tumours, and delivered in the pre-operative setting with an increased risk of post-operative wound complications. These patients have higher risks of metastatic disease. Can the development of early metastatic disease be predicted to re-define the role and timing of radiotherapy?

Methods: A retrospective study was conducted on patients receiving pre-operative radiotherapy at The Royal Marsden Hospital for sarcoma between 2012-2015 to determine whether blood parameters in patients who received pre-operative radiotherapy influenced the risk of disease relapse.

Results: Twenty-four patients had complete pre-op RT blood tests, and 92% were grade 2/3.

Median follow-up was 28.5 months (5-54 months) with median time to metastasis at 7 months. Anaemia (Hb <130 g/L) was demonstrated in 6 (25%) patients; elevated C-reactive protein (<10 mg/L) in 7 (29.2%); raised LDH (>192 U/L) in 3 (12.5%). Median progression-free survival for: 1. Anaemia vs normal Hb was 7 vs 16 months, 2. Elevated CRP vs normal CRP was 31 vs 10 months, 3.

Elevated LDH vs normal LDH was 5.5 vs 14 months.

Discussion: Alongside tumour subtype and grade, additional risk factors for disease relapse identified could include anaemia and high LDH. A larger prospective cohort evaluation is required to determine if these factors

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U.K; Adesegun Abudu - Professor Consultant in Orthopedic Oncology and Reconstructive Surgery / Royal Orthopaedic Hospital - Birmingham, U.K.

can predict early distant relapse to provide an â&#x20AC;&#x2DC;at-riskâ&#x20AC;&#x2122; score and negate the role of radiation. This may help to determine the timing of radiotherapy and intensity of follow-up for these high risk patients.

DAY ONE - SESSION: 15:15 - 16:18 Prediction of recurrence after sarcoma treatment. Can it help plan follow up?

Robert Grimer, Michael Parry, Lee Jeys; Royal Orthopaedic Hospital, Birmingham Early detection of local recurrence or metastatic disease is one of the principal reasons why we follow patients up following sarcoma treatment. Knowledge of the risk of developing either of these events at various time periods may help clarify both the frequency and the modality of follow-up.

This study looks at a large cohort of patients with sarcomas to identify risk factors in both the short and long-term for recurrence.

Method: All patients treated for at a sarcoma centre with newly diagnosed bone or soft tissue sarcomas, no metastases at diagnosis and adequate follow-up were included in the study.

Results: 5958 patients were included in the analysis of which 51% had a STS and 49% a bone sarcoma. 2981 patients (50%) developed metastases at a median time of 1.25 years. Risk factors for the development of metastases were: high-grade (HR 4.2), amputation (versus limb salvage) (HR 1.8), involved margins (HR 1.5), male sex (HR 1.1), older age (HR 1.013) and size of tumour (HR 1.03).

6926 patients were available for the local recurrence study of whom 14% developed a local recurrence. Risk factors were margins, grade, limb salvage, age and tumour size.

Using these criteria for both local recurrence and metastases it is possible to create a model to predict the risk of developing

either local recurrence or metastases. For high risk patients, most metastases (95%) developed within five years but in low risk patients more than half the metastases developed after five years. 88% of those who develop metastases will die of their disease with a median survival of 10 months.

In patients who are alive at five years without metastases or local recurrence (1953 patients) 218 (11.2%) subsequently died of their disease. Further survival with tumour-related death as the endpoint was 91.4% at 10 years and 87% at 15 years. The only factors related to survival were age, high-grade tumour and further development of local recurrence.

Conclusion: This study has been done to try and identify risk factors for recurrence and help start a discussion about how we follow patients up.

Most local recurrences are still detected by the patient and models should be developed to train patients to look better for this.

Most metastatic disease still proves fatal and the value of early detection remains debatable. Stratified follow-up based on risk factors may well be worth considering and computer models could easily be developed to identify a patientâ&#x20AC;&#x2122;s individual risks at a given time period.

DAY ONE - SESSION: 15:15 - 16:18 A multi-centre, retrospective study of 408 retroperitoneal, abdominal and pelvic schwannomas: Recommendations for management & surveillance

James Monteiro de Barros, Samuel Ford, Queen Elizabeth Hospital, Birmingham O Rintoul-Hoad (1), M Chetan (1), R Massey (2), A. Gronchi (3) & On Behalf of Contributing TARPSWG Centres: Royal Marsden, Milan, Amsterdam, Toronto, Ottawa, Emory & Mayo Cllinic, Florida (1) Queen Elizabeth Hospital, Birmingham (2) University of Durham (3) Istituto Nazionale dei Tumori, Milan Aims

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Methods Demographic, radiological, histological and clinical data was collected from 408 patients presenting to 8 centres from 2000-16. To measure the rate of growth, the date and dimensions of schwannoma found on CT/ MRI was recorded, with schwannomas treated as an ovoid, volumes calculated with V=4/3πabc. Recurrence/Residual disease was recorded following R0/R1 and R2 resections to determine the role of post resection surveillance.

Results There is a mean growth rate of 18.4% per year (n=171), with growth > 100% (n=9).

The number of resection was 248/408 (60.8%), with no malignant transformations noted. 217 had R0/R1 resections with 5/98 undergoing surveillance demonstrating recurrence. 22 had R2 resections with 13/19 demonstrating residual/recurrent disease. 9 resections had no margins recorded.

Conclusions If symptomatic: offer resection. If not symptomatic: for repeat scan in 1 year.

If small and no rapid growth on repeat scan: repeat scan in 1 year. If there is still no change in growth rate, then able to discharge. If large & likely to become symptomatic surveillance/operative management.

DAY ONE - SESSION: 15:15 - 16:18 Efficacy of olaratumab, a human anti–platelet-derived growth factor α (PDGFRα) monoclonal antibody, in combination with doxorubicin (Dox) vs Dox alone, in advanced soft-tissue sarcoma (STS) – a subanalysis of the first line population in the JGDG study

Robin L. Jones, The Royal Marsden Hospital, London, UK; Nora Drove, Eli Lilly and Company, Madrid, Spain; Pratibha Suresh, Eli Lilly and Company, Basingstoke, UK; Alan J. Lenox-Smith, Eli Lilly and Company, Basingstoke, UK; Patrick M. Peterson, Eli Lilly and Company, Indianapolis, IN, USA; Scott S. Barker, Eli Lilly and Company, Indianapolis, IN, USA Introduction Olaratumab has been approved by EMA in combination with Dox for anthracyclinenaïve, adult, advanced STS not amenable to curative treatment with radiation/surgery.

Methods A phase 1b/2 study was conducted, open‑label, randomised phase 2 of Dox+/olaratumab in unresectable/metastatic STS (NCT01185964, Tap et al. 2016). Patients received Dox (75mg/m2 day 1, every 21 days) +/- olaratumab (15mg/kg days 1 and 8, every 21 days) for up to 8 cycles.

After 8 cycles of Dox+olaratumab, patients could continue olaratumab monotherapy until disease progression or unacceptable toxicity. Results of pre-specified subgroup analyses by number of prior therapies for metastatic disease (0 vs ≥1) are reported using Cox and Kaplan-Meier methods for data analyses.

Results Of 133 patients randomised in the JGDG study, 87 (65%) were first line. In the Doxarm (n=47, 54%), patients had a median PFS of 4.1 months (95% confidence interval [95%CI]) [2.8-6.2], while patients treated with Dox+olaratumab (n=40, 46%) had 6.6 months [3.1-9.3] (unstratified HR: 0.77 [0.48‑1.25]; p=0.28). The median OS was 14.7 months [8.0-18.7] in the Dox-arm and

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Due to their rarity, the management of schwannomas is unclear, with some centres undertaking multiple resections to others maintaining long-term surveillance. The project rationale is to exploit clinical, radiological and histopathological parameters to determine if: a) low risk schwannomas may be followed up with short duration surveillance b) schwannomas of higher risk +/- patient factors necessitate resection c) there is a role of prolonged radiological surveillance post resection.

The primary aim is to create a predictive tool to guide management (observation or resection).

29.1 months [16.3-NE] for Dox+olaratumab (HR=0.47 [0.27- 0.81]; p=0.0051). Patients with ≥1 line had a median PFS of 5.4 [1.6-5.6] and OS of 13.2 [8.2-21.7] in the Dox-arm (n=20, 43%) and median PFS of 6.5 [2.8-8.3] and OS of 22.7 months [15.8-31.7] for Dox+olaratumab (n=26, 57%; HR=0.41 [0.19-0.92], p=0.027 and HR=0.55 [0.281.10], p=0.09, respectively).

Conclusion The efficacy in patients treated in first line with Dox+olaratumab is comparable to the efficacy in patients treated in first and later lines combined.

DAY ONE - SESSION: 15:15 - 16:18 A national collaboration to develop a sarcoma-specific patient report outcome measure: Phase 1 - Exploring patient experiences

Ana Martins, University College London Hospitals NHS Foundation Trust; Dr Rachel M Taylor and Professor Jeremy S Whelan, University College London Hospitals NHS Foundation Trust; Lesley Storey (Queens University Belfast); Mary Wells (Imperial College Healthcare NHS Trust); Lorna A Fern (National Cancer Research Institute); Lindsey Bennister (Sarcoma UK); Craig Gerrand (The Newcastle-upon-Tyne Hospitals NHS Foundation Trust); Maria Onasanya (patient representative); Julie Woodford (The Royal National Orthopaedic Hospital); Rachael Windsor (University College London Hospitals NHS Foundation Trust) Background Introducing patient-reported outcome measures (PROMs) into clinical practice is known to improve patient-clinician communication, patient experience and outcomes1. While there are many generic cancer PROMs there are none developed for sarcoma so these may not capture issues that are tumour-specific.

Aim To describe the experiences of being diagnosed, treated and living with sarcoma

to inform the development of a PROM. Method Participants across the UK were recruited by healthcare teams or through charities.

Due to the heterogeneity of sarcoma, recruitment considered biometric factors, location of care, sarcoma type, treatment intent, treatment type, time since diagnosis, and other factors. 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 soft tissue (62%), bone (28%) and GIST (10%)]. Five overarching themes were identified: impact of the diagnostic timeline (e.g. time to diagnosis; professionals’ roles), physical wellbeing (e.g. mobility), emotional wellbeing (e.g. fear of recurrence), social wellbeing (e.g. peer support) and financial wellbeing (e.g. challenges). The analysis identified the core experiences common to all sarcoma patients and the variances in reported experience depending on factors such as age and sarcoma type.

Conclusion In the first step towards developing a sarcoma-specific PROM we have identified the key defining characteristics of patients’ sarcoma experiences. While some were similar to experience in other cancer types, many were unique to this population.

These will form the basis for content to be included in the PROM. 1.Greenhalgh J. The application of PROs in clinical practice: what are they, do they work and why? Quality of Life Research, 2009, 18:115-123 DAY ONE - SESSION 15:15 - 16:18 Magnetic Nanoparticle Bearing Mesenchymal Stem Cells For Magnetic Hyperthermia Treatment of Sarcomas

Fiano Kerans, University of Edinburgh;

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The use of magnetic hyperthermia (MH) therapy for a number of difficult to treat cancer types such as glioblastoma is gaining interest and has the potential to be used in the management of primary, recurrent and metastatic sarcoma. A major problem limiting the clinical use of MH is uptake, retention and toxicity of manufactured magnetic nanoparticles (MNPs) and the inability to adequately and optimally localise MNPs at the tumour site. We have developed a novel methodology that results in mesenchymal stem cells (MSCs) intrinsically producing magnetic nanoparticles (MNPs). The aim of this proof of concept / pilot study was to demonstrate that application of an alternating magnetic field (AMF) to mesenchymal stem cells (MSCs) which are intrinsically producing MNPs results in a MH response killing both the MNP-bearing MSCs and co-cultured sarcoma cells. In vitro studies demonstrate that application of alternating magnetic field (AMF) to MSCs transfected with a gene that results in the production of intracellular magnetic nanoparticles (mms6-MNPMSCs) either alone or cultured with an osteosarcoma cell lines result in significant cell death (>50%) of both the MSCs and tumour cells when compared to application of AMF in the absence of magnetic nanoparticles MNPs (~12%). These results support the idea that MSC’s induced to produce MNPs may contribute to magnetic hyperthermia treatment of patients with sarcoma.

DAY ONE - SESSION: 15:15 - 16:18 Is Denosumab an Effective Treatment for Giant Cell Tumor of Bone in the Pelvis and Sacrum?

Michael Parry, Royal Orthopaedic Hospital; Robert Grimer, Royal Orthopaedic Hospital Andrea Sambri – Istituto Ortopedico Rizolli - Italy; Manuel R Medellin – Royal Orthopaedic Hospital - U.K; Laura

Campanacci – Istituto Ortopedico Rizolli Italy; Costantino Errani - Istituto Ortopedico Rizolli - Italy; Davide Maria Donati – Istituto Ortopedico Rizolli - Italy; Czar Louie Gaston - Royal Orthopaedic Hospital - U.K; Michael Parry - Royal Orthopaedic Hospital - U.K; Robert Grimer - Royal Orthopaedic Hospital - U.K. BACKGROUND The aim of this paper is to evaluate the use of Denosumab in pelvic/sacrum giant cell tumors of bone (GCTB).

PATIENTS AND METHODS: We retrospectively reviewed a cohort of 26 patients with aggressive GCTB in pelvis or sacrum treated with Denosumab at two referral orthopaedic oncology centres in Europe. Clinical response and local recurrence were recorded and the radiologic responses were evaluated with the MD Anderson criteria.

RESULTS: 69% of the pelvic GCTB treated with Denosumab presented partial or good radiologic responses (type 3 or type 4) after 49 weeks of treatment. Denosumab was administered as adjuvant therapy prior and after surgery in 11 patients (group A), and as the only treatment without any surgical intervention in the other 15 patients (group B). In group A, 62% of local recurrence was observed in patients treated with intralesional curettage. No recurrences were identified after en bloc resection. In group B, 9 patients were on continuous bimonthly long term Denosumab administration with type 3 and 4 responses after a mean of 251 weeks of treatment. Six patients stopped Denosumab and 4 of them (66%) remained radiologically unchanged after 10 months of follow up CONCLUSIONS: Long-term Denosumab therapy can be considered with curative intent for pelvic and sacrum GCTB. The administration interval may be increased in patients presenting adequate radiological responses.

Patients with inadequate radiological

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Donald Salter, University of Edinburgh; Lisa Lungaro University of Edinburgh; Asim Azfer University of Edinburgh

responses have a higher risk of local relapse after Denosumab stoppage. If surgical intervention is required wide resection may be advisable to reduce the risk of recurrence.

DAY ONE - SESSION: 15:15 - 16:18 Soft Tissue Sarcoma In Dogs: What Can We Learn From Manâ&#x20AC;&#x2122;s Best Friend?

test the hypothesis that cross-talk between the tumour and the stroma is important in sarcoma behavior. Using the results of these studies, opportunities for using this comparative disease model will be discussed, with the goal of improving prediction of tumour behaviour and testing new treatment modalities.

DAY ONE - SESSION: 15:15 - 16:18 Bone scintigraphy vs whole body MRI in the staging of Ewing sarcoma of bone: a 10-year review

Jonathan Bray, Fitzpatrick Referrals Oncology and Soft Tissue Soft tissue sarcoma (STS) are common in the dog, representing almost 15% of all subcutaneous tumours. While many dogs have good outcomes, one in five patients may die of their disease despite surgery.

Local recurrence is the most common event following surgery, occurring in between 7-75% of patients. Higher rates of recurrence are reported with high-grade tumours and/or when surgical margins about the tumour have been inadequate.

Tumour recurrence is consistently associated with reduced overall survival and tumourrelated death.

The biologic and clinical behaviour of canine STS share many similarities with their human counterpart. As with the human disease, the precise surgical margins required for adequate tumour control are unknown and tumours located on the limbs can often preclude wide surgical margins being obtained without amputation.

In both species, the pseudocapsule that surrounds the sarcoma is an important zone of nascent and evolving neoplastic activity.

Understanding the individual characteristics of this interface with the host may help improve prognostication and treatment strategies.

In this presentation, the author will detail the current status of sarcoma management in the dog, and outline the many similarities to the human disease. To date, the investigators have assessed the role of several extra-cellular matrix molecules to

Sarah Kalus, Royal National Orthopaedic Hospital; Asif Saifuddin, Royal National Orthopaedic Hospital Aim: To compare bone scintigraphy and whole body MRI in the staging of Ewing sarcoma.

Methods: All patients with a histological diagnosis of Ewing sarcoma made at the Royal National Orthopaedic Hospital between January 2007 and December 2016 were included in this retrospective study. Analysis included age range and mean age, sex, and skeletal staging including bone scintigraphy and whole body MRI. Results: 140 patients were diagnosed with Ewing sarcoma, with an age range of 2-56 years, mean age of 18.6 years, and M:F ratio of 2:1.

107 patients underwent bone scintigraphy, of whom 19 (17.8%) had lesions consistent with metastases. Whole body MRI was performed in 75 patients, demonstrating evidence of bone metastases in 20 cases (26.7%). 67 patients were investigated with both bone scintigraphy and whole body MRI. Of these, 17 (25.4%) had evidence of bone metastases. In 13 of these patients (76.5%), bone metastases were detected on both studies, while 4 patients (23.5%) were considered to have bone metastases on whole body MRI alone, with bone scintigraphy revealing no evidence of metastatic disease.

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6% of patients (2/33) had been screened for HBV; this had increased to 72% (31/43)

by October 2017. Of the patients not screened, 11/12 were established on Imatinib prior to August 2016 and eight have now discontinued treatment. Eight of nine patients commencing Imatinib after 1/8/2016 (89%) underwent full HBV screening within 91 days of commencement.

DAY ONE - SESSION: 15:15 - 16:18 The development of a Hepatitis B (HBV) screening programme for patients with Gastrointestinal Stromal Tumours (GIST) on Imatinib treatment - A Local Experience at Guy’s and St Thomas’ NHS Foundation Trust (GSTT)

Of those screened, 13% (4/31) had prior exposure to HBV. A further 6% (2/31) had active infection; this was much higher than that observed in sentinel surveillance (1.4% in London).1 Subsequently, guidelines were developed for management of these patients. All went on to receive antiviral treatment with no evidence of viral reactivation to date.

Magdalene Joseph, East and North Hertfordshire NHS Trust; Sarah Ngan, Guy’s and St Thomas’ NHS Foundation Trust; Jonathan Helbrow, Verity Williams, Nicholas Maisey, Iona Coltart, Alex Robson, Terry Wong Guy’s and St Thomas’ NHS Foundation Trust (all)

Conclusion In our cohort, a significant proportion of patients have latent or active HBV. Retrospective testing proved challenging, but prospective screening rates are satisfactory, and antiviral therapy is instigated and monitored effectively.

Introduction In April 2016, a manufacturer’s alert was issued highlighting the risk of HBV reactivation with BCR-Abl tyrosine kinase inhibitors, recommending HBV screening prior to commencing treatment. However, this was not standard of care at GSTT. We undertook a quality improvement project (QIP) to improve HBV screening for these patients.

Reference 1) Public Heath England. Annual report from the sentinel surveillance study of blood borne virus testing in England: Supplementary tables for January to December 2015. Health Protection Report.

2016. 10 (24)

Methods All patients receiving Imatinib for GIST between August 2016 and October 2017 were included. Multiple QIP cycles were undertaken during this period to evaluate HBV screening rates. Interventions included highlighting untested patients in pre-clinic meetings, educating the multi-disciplinary team and implementing e-noting prompts.

Results Forty-three patients received Imatinib during this period. In August 2016, only

DAY TWO - SESSION: 10:00 - 10:45 Incidence and survival of bone and soft tissue sarcoma (STS) in England between 1996 and 2015: An analysis from the National Cancer Registration and Analysis Service (NCRAS)

Kwok Wong, Public Health England Sandra Strauss, University College London Hospital (UCLH); Fatima Isa, Public Health England; Sarah McDonald, Sarcoma UK; Pesheya Doubleday, Public Health England; John Broggio, Public Health England; Luke Hounsome, Public Health England; Martine Bomb, Public Health England;

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Conclusion: Accurate staging is critical in the management of Ewing sarcoma. Both bone scintigraphy and whole body MRI are commonly performed for staging Ewing sarcoma of bone. Whole body MRI is however more sensitive and can replace bone scintigraphy in the imaging work-up of patients with Ewing sarcoma.

Background: Sarcomas are rare tumours representing 1% of cancers. Accurate, contemporary national data is valuable to patients, clinicians, service providers, patient advocacy groups, charities and researchers. NCRAS collects data on all patients in England diagnosed with cancer but analysis is demanding due to small numbers, complex pathways and the heterogeneous nature of disease.

Methods: NCRAS data was analysed to determine incidence and survival of patients with sarcomas between 1996 and 2015. Incidence was reported per 100,000 person-years.

Kaplan-Meier 5-year overall survival was reported according to sarcoma subtypes and changes across 5 –year aggregated cohorts.

The log rank test was used to compare the survival trend between periods.

Results: Between 1996 and 2015, an average of 514 (range: 442-577) and 3107 (range 24883766) patients were diagnosed per year with bone and soft tissue sarcoma (STS) respectively. Between 1996-2001 and 20112015, five–year survival improved for bone sarcomas from 46% to 63% (p<0.0001) and STS from 47% to 56% (p<0.01). Notably, an improvement in survival was observed for patients with osteosarcoma between 2001-2005 and 2011-2015 from 36% to 50% (p<0.01). Angiosarcoma had the poorest five-year survival at 29% for 2011-2015 and no improvement since 1996 (p=0.07).

Conclusion: This is the first national population analysis to demonstrate an improvement in survival for bone and STS over the last two decades.

Further analysis is required to determine factors influencing outcome and work is ongoing as part of the ‘Get Data Out’ initiative to continue to provide current data to the sarcoma community.

DAY TWO - SESSION: 10:00 - 10:45 Treatment and outcomes of borderline

and malignant Phyllodes tumours of the breast

Gargi Kothari, Royal Marsden Hospital; Aisha Miah, Royal Marsden Hospital; Omar Almuderis, Clare Peckitt, Shane Zaidi, Gillian Ross, Andrew Hayes, Dirk Strauss, Charlotte Benson, Robin Jones, Emma Kipps Organisation: Royal Marsden NHS Foundation Trust, London, UK Background Phyllodes tumours (PT) are uncommon fibroepithelial lesions for which optimal management remains unclear.

Methods This single institution study reports on patients with borderline and malignant PT referred between 2000 and June 2017.

Patients were identified from a prospective database. Kaplan-Meier overall survival (OS) was determined and univariate analyses performed. Local recurrence(LR) and distant metastasis(DM) rates are reported.

Results Of 82 patients referred, 70(85%) had malignant PT (2 with previous benign PT).

Median age was 50years (range 18–86).

Mean tumour size was 8cm (range 0.942cm). Three patients had synchronous lung metastases. Local excision was performed in 33(42%) and mastectomy in 45(58%) patients. Margins were >1cm in 29(47%) patients. Eighteen(25%) received adjuvant radiotherapy, of whom 1(6%) had LR. One patient had adjuvant chemotherapy.

Sixty-six patients with initially localised disease and >3months follow-up were included in outcome analyses. Median OS was 174months and greater for patients <50years (not reached [NR] vs.

90months, p=0.022) and right-sided PT (NR vs. 174months, p=0.043). Sixteen(23%) patients had LR, of whom 9(56%) had local excision, 1(6%) had adjuvant radiotherapy, and 7(44%) developed DM. Patients who received adjuvant radiotherapy were significantly more likely to have malignant PT (100% vs. 80%, p=0.038), tumour

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Conclusions Management of PT was inconsistent, highlighting the need for national guidelines. PT have high rates of LR, which may be reduced by adjuvant radiotherapy.

DAY TWO - SESSION: 10:00 - 10:45 Diagnostic Accuracy of Percutaneous Biopsy in Retroperitoneal Sarcoma

Hannah Tattersall, University Hospital Birmingham; Max Almond, University Hospital Birmingham; Fabio Tirotta, Carlo Morosi, Alessandro Gronchi, Marco Fiore (all Istituto Nazionale dei Tumori, Milan) Background: Although surgery is the mainstay of treatment in RPS, neoadjuvant strategies are increasingly being considered in high-grade tumours. The aim of this study was to analyse the safety and accuracy of percutaneous biopsy (PB) in RPS with respect to both histological type and tumour grade.

leiomyosarcoma (90%), and lowest in welldifferentiated liposarcoma (WDLPS) (49%) due to under-recognition of DDLPS in this cohort. Overall agreement between tumour grade on biopsy and final pathology was 60% (kappa 0.640). The PPV of a biopsy reporting a high-grade malignancy was 85%, with lower concordance between biopsy and final pathology in low and intermediate grades. In one case a “benign” biopsy was found to be a high-grade malignancy on final pathology. The ability to predict high grade tumours on biopsy was good (ROC curve analysis: 0.795; 95% CI: 0.722 – 0.868, p<0.001). There was no PB-related seeding or mortality in this cohort.

Conclusions: PB in suspected RPS is safe and informative, with a high PPV in DDLPS and LMS. PB may misdiagnose DDLPS as WDLPS in one half of patients, and grade 3 tumours as grade 2 tumours in 15%.

DAY TWO - SESSION: 10:00 - 10:45 MRI Differentiation of Low-grade from High-grade Pelvic Chondrosarcoma

Dr Nilesh Gurjar, Royal National Orthopedic Hospital, Stanmore; Dr Asif Saifuddin, Royal National Orthopedic Hospital, Stanmore

Methods: Patients with primary RPS who underwent PB and resectional surgery between 2005 and 2016 at two tertiary European centres in Milan and Birmingham were identified.

Diagnostic accuracy of PB was established by correlating biopsy diagnoses with postoperative pathology. Data on biopsy-related morbidity and tumour seeding were also evaluated.

Results: 227 patients (162 Milan and 77 Birmingham) were included in the PB accuracy analysis.

Histological subtype was concordant on biopsy and final pathology in 67% of cases (Kappa 0.606). The positive predictive value (PPV) of PB was high in dedifferentiated liposarcoma (DDLPS) (97%) and in

Objectives: To identify the MRI features which differentiate low grade (grade 1) from high grade (grade 2, 3 and dedifferentiated) pelvic chondrosarcoma.

Methods: We identified all patients treated for pelvic chondrosarcoma over a period of 10 years (2007-2016). MRI studies were assessed for bone marrow oedema, soft tissue oedema, cortical destruction/soft tissue mass, joint involvement, distant metastasis and tumour volume and were compared with needle biopsy results and post-operative histology.

Results: The study included 52 patients, 37 male and 15 female with a mean age of 58

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size >5cm (87% vs. 53%, p=0.021) and mastectomy (83% vs. 54%, p=0.026), and less likely to recur locally (6% vs. 28%, p=0.046). Thirteen(19%) patients died from metastatic PT (12 had malignant PT).

(range 27-84 years) were included in this study. Final histological diagnosis was 12 grade 1, 24 grade 2, 12 grade 3 and 4 dedifferentiated chondrosarcomas; a total of 12 low-grade and 40 high-grade lesions.

8% of low-grade and 50% of high grade chondrosarcomas showed bone oedema, 92% of low-grade and 93% of high-grade chondrosarcomas showed extra-osseous extension, soft tissue oedema was seen in 50% of the low-grade and 90% of the highgrade chondrosarcomas, joint involvement was seen in 25% of the low-grade and 53 % of the high-grade chondrosarcomas, and features of dedifferentiation were seen in 20% of the high-grade chondrosarcomas.

Mean tumour volume for grade 1 tumours was 1139cm³ and 1196cm³ for high-grade tumours.

Conclusion: On MRI, bone marrow oedema, soft tissue oedema, joint involvement and features of dedifferentiation can potentially differentiate low-grade from high-grade pelvic chondrosarcomas.

Methods Using the National Cancer Registry Ireland (NCRI) database, all patients diagnosed with a sarcoma age 0-24 between 19942014 were identified. Survival was based on matching of cases against national death certificate data complete up to 31 December 2014 & grouped in 2 diagnostic cohorts: 1994-2003 and 2004-2014 and examined according to the ICCC. Results 577 patients less than 25 years were diagnosed with a sarcoma between 19942014; 321 under 15. Significantly poorer survival was noted for the AYA patients compared with paediatric patients for the following sarcoma groups and diagnosis periods Malignant bone tumours, 1994-2013 (EHR 1.62, 1.05-2.48, P=0.026) and 1994-2003 (EHR 1.87, 1.05-3.33, P=0.032);

DAY TWO - SESSION: 10:00 - 10:45 Survival of paediatric and adolescent / young adult (AYA) cancer patients with sarcoma in Ireland during 1994-2014: comparisons by age

Ewing & related tumours), 1994-2013 (EHR 2.04, 95% CI 1.11-3.72, P=0.021) and 19942003 (EHR 2.68, 95% CI 1.22-5.87, P=0.014);

Dr Scheryll Alken, St James’s Hospital; Dr Deirdre O’Mahony, Cork University Hospital; Dr Charles Gilham, St Luke’s Radiation Oncology Network; Dr Cormac Owens, Our Lady’s Children’s Hospital Crumlin; Dr Cliona Grant, St James’s Hospital; Dr Sandra Deady, National Cancer Registry Ireland; Dr Michael Capra, Our Lady’s Children’s Hospital Crumlin; Dr Jane Pears, Our Lady’s Children’s Hospital Crumlin; Dr Paul Walsh, National Cancer Registry Ireland; Dr Deirdre O’Mahony, Cork University Hospital Some studies indicate that survival of AYAs with cancer may be inferior to that of younger children with similar cancers, possibly related (in part) to differences in access to centralised or standardised

treatments. We sought to examine the comparative survival of paediatric & AYA patients in Ireland across a 20-year period.

Soft tissue sarcomas), 1994-2013 (EHR 2.14, 1.29-3.53, P=0.03) and 2004-2013 (EHR 2.52, 1.20-5.25, P=0.014).

Conclusions This study highlights the disparities that exist in outcomes for AYA patients with sarcomas treated in Ireland. The exact cause for this is unclear and is likely multifactorial, possibly owing to lack of standardised/ centralised services. Future development on a national level is imperative.

DAY TWO - SESSION: 10:00 - 10:45 The ITAP project – successful or not?

Robert Grimer - Royal Orthopaedic Hospital; Steven Cannon - Royal National Orthopaedic Hospital, Stanmore; Will Aston Page 38

The ITAP (Intraosseous Transcutaneous Amputation Prosthesis) study was a clinical study aimed at improving outcomes for patients with above knee amputations.

The study ran from 2008 to 2016 and the objective was to evaluate the safety and feasibility of attaching a prosthetic limb using ITAP as well as assessing quality of life changes and patient acceptability.

Methods The study involved two centres in the UK (RNOH and ROH) with 20 patients and a minimum follow up of 18 months. Ethical approval was obtained. Due to a high incidence of infection in the first 10 patients the ITAP device was modified to incorporate silver for the next 10 patients.

Results At 18 months follow up 80% of patients had satisfactory skin assessments around the ITAP implant. However superficial infection was commonplace and in 3 patients led to explantation of the implant.

Of the remaining 17 patients, 11 are on either continuous low dose or intermittent antibiotics. All patients however reported significantly improved function and QoL. Conclusion The ITAP device has shown very high patient satisfaction and functional improvement in patients who have problems with limb fitting after an above knee amputation. Infection around the implant where it penetrates the skin is however an unresolved problem.

Further work is still required to identify a reproducible method of an implant penetrating the skin to allow fitting of a prosthesis.

DAY TWO - SESSION: 10:00 - 10:45 Centralising Sarcoma Services in Ireland

Orlaidh Burke, SLRON, Dublin; Margaret Brennan, SLRON, Dublin; Chalres Gilham, SLRON, Dublin Centralisation of treatment of sarcoma is essential for improving patient outcomes.

NICE recommend that all patients with a confirmed diagnosis of sarcoma should have their care supervised by a sarcoma MDT. On average there are 176 new cases of sarcoma diagnosed annually in Ireland.

Significant progress has been made to centralise adult sarcoma services in Ireland with the formation of two national sarcoma MDTs (St Vincent’s University Hospital, Dublin and Cork University Hospital, Cork).

Since it’s inception in 2013 (until July 2017), 342 patients with a confirmed diagnosis of sarcoma have been discussed at the Dublin MDT. Of these 20 were diagnosed and referred in 2014 and 108 in 2016 highlighting the enhanced awareness of the service. 168 (49.1%) were female and 174 (50.9%) were male with a mean age of 55 years. There have been 288 (84.2%) soft tissue and 54 (15.8%) bone tumours.

Amongst the soft tissue sarcomas there were 76 (26.4%) liposarcomas, 46 (16%) leiomyosarcomas, 42(14.6%) fibroblastic sarcomas, 18 (6.3%) GIST, 17 (5.9%) malignant peripheral nerve sheath tumours (MPNST), 15(5.2%) synovial sarcoma, 7(2.4%) rhabdomyosarcoma, 6(2%) angiosarcoma.

The remaining subtypes made up 21.2%.

Within the bone tumours 18(33.3%) were giant cell tumours, 17(31.4%) osteosarcomas, 11 (20%) chondrosarcomas and 9 (16.7%) Ewing’s sarcoma. 46(13.5%) cases were metastatic at first MDT discussion. 80% of cases were referred from hospitals within Dublin. In order to manage the increasing referrals there is an pressing need for further investment in diagnostic and clinical services.

DAY TWO - SESSION: 12:00 - 12:30 Circulating nucleic acid characteristics of non-metastatic STS patients

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Abstracts - Free Papers

- Royal National Orthopaedic Hospital, Stanmore; Tim Briggs - Royal National Orthopaedic Hospital, Stanmore; Paul Unwin - Stanmore Implants (Now part of Stryker); Gordon Blunn - Dept of Biomedical Engineering, UCL

Nicholas Eastley, The University of Leicester; Associate Professor Robert U Ashford, East Midlands Sarcoma Service; Barbara Ottolini - The University of Leicester; Rita Neumann The University of Leicester; Tom McCulloch Nottingham University Hospitals NHS Trust; Jacqui Shaw - The University of Leicester; Nicola Royle - The University of Leicester

ctDNA as a source of novel STS biomarkers, the complex alterations present in STSs pose several challenges to this field. More work investigating STS patient ctDNA is required, which should consider investigating circulating tumour derived copy number alterations.

Background A key barrier to improving STS patient outcome is a lack of circulating biomarkers.

Short fragments of DNA can be found in the circulation where they are termed cell free DNA (cfDNA). In cancer patients a proportion of cfDNA is tumour derived (circulating tumour derived DNA or ctDNA).

In several cancers ctDNA characteristics have been shown to correlate with disease progression or recurrence. We aimed to investigate the cfDNA and ctDNA characteristics of a cohort of non-metastatic STS patients throughout follow up.

DAY TWO - SESSION: 12:00 - 12:30 A Phase I Study of the Poly-ADP Ribose Polymerase (PARP) inhibitor, Niraparib (NIR) in combination with Temozolomide (TMZ) in patients with advanced incurable Ewing Sarcoma (ES): Final results of SARC025 Arm 1.

Methods Intra-operative and serial post-operative plasma samples were collected from 22 patients undergoing attempted curative STS resections. Total cfDNA levels were calculated using qPCR and evidence of ctDNA sought using either a custom designed semiconductor sequencing chip or mutation/patient specific droplet digital PCR assays. All cfDNA/ctDNA characteristics were correlated with clinical outcome measures including recurrence and survival.

Results Total cfDNA levels did not correlate with perioperative disease burden or the development of recurrence. Evidence of intra-or post-operative ctDNA was identified in 7/22 patients. In only one case did ctDNA levels rise consistently towards the development of radiological detectable recurrence.

Conclusions Circulating tumour derived DNA can be detected in STS patients that have undergone attempted curative treatment.

Although this highlights a potential role for

Sandra J Strauss, UCL Cancer Institute and University College London Hospitals NHS Trust; Rashmi Chugh, University of Michigan; Karla V Ballman, Weill Cornell Medical College; Lee J Helman, Children’s Hospital of Los Angeles; Brigette C. Widemann, National Cancer Institute; Douglas S. Hawkins, Seattle Children’s Hospital; Leo Mascarenhas, Children’s Hospital of Los Angeles; John W. Glod, National Cancer Institute Birgit Geoerger, Institut Gustave Roussy; Jeremy Whelan, University College London NHS Trust; Denise Reinke, SARC; Shreyaskumar Patel, University of Texas MD Anderson Cancer Center Objective: PARP inhibitors demonstrated potential therapeutic value in pre-clinical models of ES, with notable synergy observed in combination with alkylating agents. We initiated a phase I study of the PARP inhibitor niraparib (NIR) with temozolomide (TMZ) to determine the dose-limiting toxicities (DLT) and maximum tolerated dose (MTD) of different schedules of the combination in pts with pre-treated ES. Methods: Eligible pts were assigned to Cohort A with continuous NIR 300 mg daily and TMZ on D2-6 of 28D cycle. If DLTs were observed within the first 3 dose levels, pts were assigned to cohort B with interrupted NIR dosing (D1-7, 1-14 or 1-21). In Cohort C,

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Results: Between 07/14-05/16, 17 eligible pts with confirmed ES were enrolled. Median age was 23 (range 13-44), and median prior therapies was 4 (range 1-10) with 16 pts receiving prior radiation. As DLTs were observed in dose level A1, the study progressed to cohort B. Five pts experienced at least 1 DLT at 3 dose levels most notably thrombocytopenia (Table 1). No DLTs occurred at final dose level C1. No responses were observed.

Pharmacodynamic parameters in tumor biopsies and blood are undergoing evaluations.

Conclusions: The MTD reached is NIR 200 mg D1-7 in combination with TMZ 30 mg/m2 D2-6 with DLTs being hematologic in nature. In view of toxicity observed and compelling preclinical data, exploration of a combination of NIR and irinotecan is ongoing in Arm 2.

DAY TWO - SESSION: 12:00 - 12:30 Management of Gynaecological Sarcomas (GS) at Pan Birmingham Gynaecological Cancer Centre (PBGCC)- A 10 year experience

Dr Lalit Pallan, Cancer Centre, Queen Elizabeth Hospital Birmingham, UK; Dr Mariam Jafri, Cancer Centre, Queen Elizabeth Hospital Birmingham, UK; Dr Jennifer Pascoe Cancer Centre, Queen Elizabeth Hospital Birmingham, UK; Mr James Nevin Pan-Birmingham Gynaecological Cancer Centre, City Hospital, Birmingham, UK; Dr Sarah Williams Cancer Centre, Queen Elizabeth Hospital, Birmingham, UK Introduction/Methods GS are a rare, heterogenous and aggressive group of cancers associated with poor prognosis in which diagnosis is complex and treatment is often complicated by

limited evidence-base. We retrospectively audited the diagnostic pathway, treatment and clinical outcomes of such patients diagnosed between 2007-2017 at the PBGCC using electronic and MDT records. Our aim was to identify areas where improvements might maximise positive patient outcomes.

Results 125 patients (56 leiomyosarcoma, 23 endometrial stromal sarcomas, 46 others) were identified from gynae-oncology MDT records with 66% also presented at specialist sarcoma MDT. Median time between discussions was 33 days (range 2-1671). 60% had definitive surgery following initial diagnosis after a median time interval of 53 days (range 10-172).

Of the 74 patientsâ&#x20AC;&#x2122; achieving macroscopic surgical clearance, 68% were placed under surveillance and 32% received adjuvant treatment. The relapse rate was 50% in the surveillance and 54% in the adjuvant treatment groups with median relapse free interval of 762 days and 915 days respectively. Median overall survival in the surveillance group was 3284 days and 1028 days in the adjuvant group. In patients presenting with advanced disease unsuitable for radical treatment (n=44), median survival was 242 days. Of these patients, 41% received systemic chemotherapy, 25% had >1 line of systemic chemotherapy and 39% received supportive care only. Other treatments included radiotherapy and hormone therapy.

Conclusions This retrospective audit has confirmed the dismal prognosis in this patient population and identified some areas within the treatment pathway that may be targets for improving patient outcomes.

DAY TWO - SESSION: 12:00 - 12:30 A National Cancer Registration and Analysis Service (NCRAS) Systemic Anti-Cancer Therapy (SACT) analysis to investigate the use of Mifamurtide - Muramyl Tripeptide Phosphatidylethanolamine (MTP-PE) in

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Abstracts - Free Papers

TMZ was to be re-escalated in 10 mg/m2/d increments from MTD determined from cohort B.

patients with non-metastatic, resectable osteosarcoma

Kwok Wong, Public Health England; Sandra Strauss, University College London Hospital (UCLH); Jackie Charman, Public Health England; Fatima Isa, Public Health England; John Broggio, Public Health England; Luke Hounsome, Public Health England Background: Methotrexate, Doxorubicin and Cisplatin (MAP) chemotherapy and surgery are standard of care for patients with localised OS. MTP-PE was found to improve survival in these patients when given in combination with post-operative multi-agent chemotherapy. In 2011, NICE approved its use for patients 2-30 years in this setting.

MTP-PE is given twice weekly for 12 weeks followed by weekly for 24 weeks (total of 48 cycles).

Aim: To understand use of MTP-PE in patients with OS across England since NICE approval.

Methods: Data was accessed through NCRAS and SACT to determine patients with OS, age, stage, surgery and chemotherapy to determine the proportion of patients receiving MTP and treatment received.

Results: Between 2012 and 2015, 60 patients received MTP-PE; average 15 annually (range 11-20) across 11 centres. Forty-two patients received MTP-PE post-operatively; in 18 there was no record of surgery and 2 were staged as metastatic. During that time, 300 patients were diagnosed with OS aged 2-30 years, 44 (16%) were designated metastatic, 138 (46%) localised and 118 (40%) stage unknown. Of those designated as localized, 110 (80%) had surgery, 75 (54%) received MAP and of those 32 MTP-PE (43%). Patients received an average of 18 of 48 (43%) cycles.

Conclusion: Fewer than 50% of eligible patients appear

to receive MTP-PE and a minority completes treatment. This data is valuable for service provision and to inform future studies.

Analysis is ongoing to better understand the data and variation in use which is required to reliably understand the impact on outcome.

DAY TWO - SESSION: 14:30 - 15:00 Identification of adult knee primary bone tumour symptom presentation: A qualitative study

Lucinda Gosling, The Royal Orthopaedic Hospital NHS Foundation Trust; Dr Alison Rushton The University of Birmingham Objectives: The aim of this study was to identify the symptom presentation of adult knee primary bone tumours from onset to Consultant diagnosis, from combined patient and healthcare professional perspectives.

Materials and methods: A qualitative study using in-depth semistructured interviews recruited a purposive sample of adult patients with a knee primary bone tumour (n = 8) and healthcare professionals with expertise in orthopaedic oncology (n = 6). Following informed consent, recorded interviews explored participantsâ&#x20AC;&#x2122; experiences of symptom presentation. A grounded theory approach was utilised to analyse transcribed data, producing themes. Methods to increase rigour and trustworthiness were employed.

Recruitment continued until data saturation was achieved.

Results: Four key themes were established: 1] Symptoms started with intermittent pain which became more severe and more constant, 2] Pain was mechanical in nature but became more difficult to ease; 3] The pain story was unusual with a protracted symptom duration and failure to improve with conservative treatment; 4] Non-painful symptoms included swelling, and systemic signs were uncommon. More similarities

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Conclusion: New insights of symptom presentation, particularly in the early stages have been provided, which are not reflected in current guidelines. Although starting similarly to routine musculoskeletal presentations, a number of distinctive features may enable earlier diagnosis.

DAY TWO - SESSION: 14:30 - 15:00 Tomotherapy based IMRT in the treatment of soft tissue sarcomas (STS), Northern Centre for Cancer Care (NCCC), 8 years experience

Jayshree Veeratterapillay and Anthony Waton, Northern Centre For Cancer Care; Dr Daniela Lee, Dr Judith Mott and Tracy Wintle, Northern Centre for Cancer Care Radiotherapy plays an important role in the treatment of soft tissue sarcomas (STS). IMRT is an advanced radiotherapy technique which enables delivery of a highly conformal dose to the target whilst sparing surrounding normal tissue. In the UK, a phase II clinical trial (IMRiS) is currently assessing the feasibility, efficacy and toxicity of IMRT in patients with bone and soft tissue sarcomas.

The NCCC was one of the first 2 NHS centres in the UK to commission a Tomotherapy planning and delivery unit. Since 2009, all STS identified at the North of England Bone and Soft Tissue Sarcoma MDT as requiring radiotherapy have been treated with Tomotherapy based IMRT, as this was considered a distinctive solution to treating long treatment fields with IMRT. Treatment target volumes and organ at risk delineation was done as per local protocol for limb sarcomas, this matched the Vortex protocol. Pre operative radiotherapy was delivered using 50Gy in 25 fractions. The

post operative dose consisted of 60Gy in 2 Gy fractions delivered in a two phase treatment. In recent years, we have delivered the post operative dose with a concurrent phase 1 and 2 technique.

We have undergone a retrospective review of patients treated using our local protocol between 2009-2017. The primary aim of this study is to assess our 5 year local control rates compared to rates from modern series.

Secondary aim is to evaluate if planning target volumes and organs at risk dose constraints from our series meet the IMRiS trial requirements.

DAY TWO - SESSION: 14:30 - 15:00 A systematic review of population-based studies examining outcomes in primary retroperitoneal sarcoma surgery

Shailen Patel, Nottingham University Hospitals NHS Trust; Catherine Walter, Nottingham University Hospitals NHS Trust; Oliver Peacock, Shailen Patel, J A Simpson, DJ Humes, CJ Walter Background: Retroperitoneal sarcomas (RPS) are rare mesenchymal tumours. Their rarity challenges our ability to understand expected outcomes. The aim of this systematic review was to examine 30day morbidity and mortality, overall survival rates and predictors of this from population-based studies for patients undergoing curative resection for primary RPS. Methods: A systematic literature of EMBASE, MEDLINE, PUBMED and the Cochrane library was performed using PRISMA for populationbased studies reporting from nationally registered databases on primary RPS surgical resections in adults. The main outcomes evaluated were 30-day morbidity and mortality and overall survival rates.

The use of additional treatment modalities and predictors of overall survival were also examined.

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between healthcare professionalsâ&#x20AC;&#x2122; and patientsâ&#x20AC;&#x2122; perceptions were found at Consultant diagnosis compared to symptom onset.

Results: Fourteen studies (n=12,834 patients) reporting from 3 national databases, which were the Surveillance, Epidemiology and End Results (SEER), the United States National Cancer Database (US NCDB) and the American College of Surgeons’ National Surgical Quality Improvement Program (ACS NSQIP), were analysed. The reported overall 30-day morbidity and mortality were 23% and 3% respectively. Reported use of neo- & adjuvant radiotherapy was 28%. No study reported loco-regional recurrence rates. Overall reported 5-year survival ranged from 52% to 62%. Independent predictors of overall survival were age of the patient, resection margin, tumour grade and size, histological subtype and receipt of radiotherapy.

Conclusion: This review of population-based data demonstrated relatively low 30-day morbidity rates in patients undergoing curative surgical resections for primary RPS. Thirty-day mortality rates were similar to other abdominal tumour groups.

There remains a paucity of data reporting recurrence rates, however 5-year survival rates ranged from 52-62%.

DAY TWO - SESSION: 14:30 - 15:00 Does the sarcoma MDT need transformation?

UK project ‘Improving the effectiveness of MDT meetings in cancer services’ highlighted inefficiencies within the MDT process. The East Midlands Sarcoma Service utilises a weekly 90-minute MDTM for soft tissue and retroperitoneal sarcoma and links five Trusts by teleconference.

Method Three different methods were employed to assess our MDTM: MDT members were surveyed with respect to content and organisation of our MDTM; a two-month period of MDTM agendas and outcomes were retrospectively analysed; member contribution to MDTM discussion was recorded in real time over a one month period.

Results Of 23 MDT members who responded, 95% wanted MDTM streamlining and there was strong agreement on the need for stricter referral criteria and the development of protocols for some patient groups. On average there were 28 patients per MDTM, allowing 3 minutes of discussion per patient.

71% were new patients and of these 23% were diagnosed as atypical lipomatous tumour or benign lesions and required discussion with no more than two MDT members. 11% of patients had decisions deferred, compared with 8% in a previous audit.

Conclusions Our MDTM requires streamlining with review of protocols and stricter policies in order to improve efficiency and concentrate our MDT resource on patients most in need. The national interest in MDTMs is likely to result in a requirement for formal MDT audit.

S. Ghorbanian, East Midlands Sarcoma Service; K. Steele, East Midlands Sarcoma Service; M.Abdelreheem, East Midlands Sarcoma Service; M.Tamimy, East Midlands Sarcoma Service; A.Raurell, East Midlands Sarcoma Service; R.Ashford, East Midlands Sarcoma Service Introduction There is currently a national drive to streamline multidisciplinary meetings (MDTMs). The Cancer Transformation Board and Department of Health project ‘Transforming MDTMs’ suggests an overreliance on MDTMs and the Cancer Research

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1. Factors influencing peopleâ&#x20AC;&#x2122;s ability to maintain their activity levels during chemotherapy for soft tissue sarcoma - a qualitative study

Sarah Dewhurst, Royal Marsden Hospital; Clare Shaw, Royal Marsden Hospital; Gill Mein - St Georges University of London Catherine Sandsund - Royal Marsden Hospital Ruth Tigue - Royal Marsden Hospital Purpose Increasing evidence suggests that physical activity can be beneficial for people being treated for advanced cancer but there is limited research within a soft tissue sarcoma population. Development of services to encourage physical activity for this group need to understand the userâ&#x20AC;&#x2122;s perspective. The aim of this study was to explore factors influencing physical activity for people undergoing chemotherapy treatment for soft tissue sarcoma.

Method Following ethical approvals, patients with capacity and a diagnosis of soft tissue sarcoma undergoing chemotherapy were recruited from a UK Cancer Centre. Following informed, written consent six individual semi-structured interviews were conducted. Data from the interviews was recorded, transcribed and thematic analysis was completed.

Results Three main themes emerged: firstly participants viewed the ability to remain active as an indicator of normality and a contributor to their overall wellbeing. Secondly participants reflected on the uncertainty of the future, both because of the daily variability in energy levels experienced after chemotherapy and also as they accepted the palliative nature of their diagnosis. Thirdly, people found it challenging to identify their need for support, resulting in further de-conditioning as their treatment progressed.

Conclusion This small study suggests the importance of physical activity is an integral part of life, enabling participation, promoting well-being and engendering a feeling of normality at a time of adjustment, loss and grief. It exposed the challenges posed by daily variability in physical ability due to fatigue and the lack of timely guidance from health professionals.

2. Indeterminate Superficial Soft Tissue Tumours - Relationship of Lesion Depth to Differential Diagnosis

Michael Khoo, Asif Saifuddin, Ian Pressney Rikin Hargunani, Royal National Orthopaedic Hospital Aim Clinically suspected soft tissue tumours may arise superficial to the fascia. Small lesions may be indeterminate by MRI evaluation. The aim of this study is to determine whether the location of a small, indeterminate soft tissue mass within the subcutaneous compartment is related to its degree of aggressiveness.

Materials and Methods All referrals to a specialist Musculoskeletal Sarcoma Service over a 12-month period with a small (<3 cm) superficial soft tissue mass, indeterminate by MRI evaluation and subsequently underwent primary excision biopsy, were included. Lesions of the fingers and toes were excluded. The lesions were categorised by their anatomical location in the subcutaneous compartment.

The histopathological diagnoses were according to WHO 2013. Statistical analysis was performed using the Chi-squared test.

Results: - 43 patients (24 males; 19 females).

Mean age 42 years (range 15-71 years). 16 lesions categorized superficial, 9 lesions central and 18 lesions deep.

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- 9 lesions were non-neoplastic, 29 benign, 1 intermediate-grade and 4 malignant.

- All intermediate-grade and malignant lesions (5/43) were located in the deep aspect of the subcutaneous compartment.

- Chi-squared analysis of histopathology (2 categories of non-neoplastic / benign and intermediate-grade / malignant) and anatomical location resulted in a p-value of 0.02.

Conclusions: The current study suggests that the location of a small, indeterminate soft tissue mass within the subcutaneous compartment may be a relevant feature in determining its degree of histological aggressiveness, with intermediate-grade/malignant lesions more likely to be located deep, adjacent to or involving the fascia.

3. Secondary Amputations For Soft Tissue Sarcoma: Recurrent Tumour Or Treatment Effect

Lynsey Green, East Midlands Sarcoma Service, Nottingham; Robert Ashford, Nicholas C Eastley - East Midlands Sarcoma Service, Leicester; Anna Raurell, Kathryn Steele, M Sarmad Tamimy, East Midlands Sarcoma Service, Nottingham; A Pabla, East Midlands Sarcoma Service, Leicester Background Disease extent of soft tissue sarcomas sometimes necessitate a primary amputation. However, there are occasions later in the disease course where an amputation becomes necessary.

our service between 2006-2017. Patients were identified from a histopathological database.

Results Eighteen eligible cases (9 male, 9 female) were identified with a wide range of STS subtypes including undifferentiated pleomorphic sarcoma (n=6) synovial sarcoma (n=5) and myxofibrosarcoma (n=3). 15 patients received an amputation for recurrent disease not amenable to limb sparing surgery (8 lower limb, 7 upper limb).

The mean number of recurrences for this group was 2.1/patient.

3 patients received an amputation to treat a complication of their initial limb sparing primary STS resection. The indications for these were infected post-irradiation pathological fracture (1), chronically infected non-healing post-radiotherapy wound (1) and chronic wound secondary to vascular insufficiency (1).

The average time between initial surgery and amputation was 28 months (range 2- 73) for those treated for recurrence and 39 months (12-69) for those treated for a complication. Only 3 patients did not receive radiotherapy as part of their primary STS management, all of which suffered local recurrence. At most recent follow up (median 53 months) 11 patients were alive without disease, 2 were alive with pulmonary metastases and 7 had died of disease.

Conclusions The majority of secondary amputations performed by our service were for recurrent tumour. Complications of treatment made up only a small proportion of our amputations.

Objectives We sought to ascertain whether delayed (secondary) amputations were due to tumour recurrence or a result of complications related to the treatment of the primary tumour Study Design &

4. Palliative radiotherapy in elderly patients with cutaneous angiosarcoma of head and neck

Methods We performed a retrospective review to investigate patients with extremity STSs that underwent an secondary amputation within

Cristiana Alzamora, Omar Al-Salihi, Lynne Coupar, Sarah-Louise Vickers, University Hospital Southampton NHS Foundation

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tissue infiltration characteristic of CA.

Introduction Cutaneous angiosarcoma (CA) is a rare malignancy with a poor prognosis that affects mainly elderly patients. Surgery followed by radiotherapy is the treatment of choice. Wide resection is difficult to achieve and comorbidity of elderly population limit the radical approach. Consequently, palliative radiotherapy appears as an option to control symptoms and tumour growth.

The aim of this study is to analyse treatment and outcomes of elderly patients who received palliative radiotherapy for CA. Materials/Methods Retrospective analysis of patients with CA of the head and neck treated with palliative radiotherapy at a single institution.

Demographic and tumour features, treatment, toxicity and outcomes were assessed.

Results Three patients (2 males, 1 female) with a mean age of 86 (range: 79-92) were included. None had lymph node or distant metastasis at diagnosis. Prevalent location was face, mean size was 10.3cm (range: 8-13cm). Two patients received 30Gy/6fractions (2fractions/week, electrons, 12mEv), and 1 received 27Gy/6fractions (2fractions/week, electrons, 8mEv). None experienced toxicity â&#x2030;Ľ grade 3. Local response was observed in all irradiated sites.

Progression outside treated field occurred in all patients, mean time 4.9 months (range: 3.9-6). Two patients developed second progression: one was marginal/in-field and other was marginal/out-field. Progression was treated with further palliative radiotherapy. None developed lymph node or distant metastasis.

Conclusions Palliative radiotherapy is well tolerate and suitable for frail elderly patient who cannot undergo radical or systemic treatment.

Despite a satisfactory local response, progression outside treated fields is a challenging pattern that reflects the diffuse

5. Validation of a novel NGS approach to improve sarcoma diagnosis using FFPE

Lauren Mc Connell, Centre for Cancer Research and Cell Biology; Prof. David Gonzalez de Castro, Centre for Cancer Research and Cell Biology; Dr. O. HoughtonBelfast Health & Social Care Trust Dr. M. Catherwood- Belfast Health & Social Care Trust Dr. JP. Stewart- Centre for Cancer Research and Cell Biology J. GazdovaCentre for Cancer Research and Cell Biology Prof. M. Salto-Tellez- Centre for Cancer Research and Cell Biology Introduction: Sarcoma diagnosis can be difficult due to the fact that there are over 60 different histological subtypes. Approximately one third of sarcoma patients harbour fusion genes or gene amplifications and molecular methods such as FISH/RT-PCR directed at these abnormalities are a useful tool in differential diagnosis. FISH/RT-PCR can however report inconclusive results in up to 25% of patients with chromosome abnormalities. Next generation sequencing (NGS) is a promising tool that could help improve the diagnosis of soft-tissue sarcomas.

Methods: A novel NGS gene capture panel containing probes for 90 fusion genes and 7 genes with frequent copy number changes was designed, optimised and validated in order to improve fusion-positive sarcoma diagnosis. Genomic DNA was extracted from 19 well-characterised FFPE samples and libraries prepared using the KAPA Hyperplus kit (Roche). Libraries were hybridised with the gene panel using the SeqCap Hybridisation kit (Roche) and sequencing performed on the NextSeq platform (Illumina).

Result: Sarcoma specific translocations were identified in 100% of previously

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Trust

characterised fusion-positive cases including 6 synovial sarcomas, 6 myxoid liposarcomas, 3 alveolar rhabdomyosarcomas, 2 Ewing sarcomas and 1 inflammatory myofibroblastic tumour. MDM2 and CDK4 amplifications were also detected in a dedifferentiated liposarcoma.

Conclusion: Our comprehensive NGS sarcoma panel using genomic DNA is at least as sensitive and specific as the combination of FISH and RT-PCR, and can be applied to clinical diagnostic specimens.

6. Heat Shock Protein (HSP27, HSP70 and HSP90) expression in Myxofibrosarcoma of the extremities –predicts prognosis?

Ayman Al Amri, Royal Liverpool and Broadgreen University Hospital; Dr Margaret Roebuck, University of Liverpool; Susha Varghese, Joseph Alsousou, Qi Yin, Coonoor Chandrasekar (Royal Liverpool and Broadgreen University Hospital) BACKGROUND: Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities with high recurrence and metastasis rate. Heat shock proteins (HSPs) are involved in cell proteostasis, and their expression by cancer cells allows the cells to survive, invade and undergo metastasis. AIMS: The purpose of this study was to investigate the HSP (HSP27, HSP70 and HSP90) in MFS of the extremities and prognosis.

HSP27 and HSP70 were highly expressed in myxofibrosarcomas, but only HSP70 showed that there was a significant relationship between HSP70 overexpression and the grade of a tumour and development of metastases and survival (P < 0.05). However, only seven (26.7 %) myxofibrosarcomas showed high expression of HSP90.

CONCLUSIONS: The findings of this study indicate that HSP70, and to a lesser extent HSP27, has an important role in MFS behaviour and may provide prognostic information for clinicians in the future. Further studies are warranted to gain more insight into the role of these HSPs in the tumorigenesis of MFS to determine whether they are possible markers of prognosis and targeting for treatment.

7. Why do we need SAM”? A narrative review of the impact of sarcoma on psychosocial wellbeing

Lesley Storey, Queen’s Univerisyt Belfast; Rachel Taylor, University College Hospital London; Mary Wells - Imperial College London Ana Martins - University College Hospital London Lorna Fern - University College Hospital London Lindsey Bennister - Sarcoma UK Craig Gerrand - Newcastle University Hospital Trust Maria Onasanya co-researcher (patient perspective) Rachael Windsor - University College Hospital London Julie Woodford - Royal Orthopaedic Hospital Jeremy Whelan - University College Hospital London

METHODS: Expressions of HSP27, HSP70 and HSP90 were analysed using immunohistochemistry (IHC) on tissue sections from 26 cases of MFSs (20 high grade, 3 intermediate grade and 3 low grade). The expression of each protein was evaluated and its relationship with clinicopathological parameters examined.

Background While there are many generic cancer PROMs these may not capture issues that are important to patients with sarcoma. There is currently no sarcoma-specific PROM. We are in the process of developing a sarcomaspecific PROM – SAM (Sarcoma Assessment Measure). As part of this multi-step process we carried out a review of the literature which we report here.

RESULTS:

Aim

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Method The literature review is being guided by search terms published previously in sarcoma and psychosocial reviews (Kwong et al. 2014; Taylor et al. 2013; Walker et al. 2016). The review includes separate consideration of qualitative and other studies.

Findings The review revealed a paucity of good quality rigorous research assessing psychosocial issues in sarcoma patients.

There was a heterogeneity of research designs with very few intervention studies.

the case of a 29year old lady who initially presented with back pain, pleuritic chest pain with a normal chest radiograph and was treated with antibiotics for a presumed chest infection. A second presentation with breathlessness and haemoptysis led to a Computed Tomography of the Chest which showed abnormal soft tissue along the pulmonary artery with aneurysmal dilatation and lymphadenopathy. Initial diagnosis was of a large vessel vasculitis for which treatment was commenced with a chemotherapeutic agent. Subsequent core biopsy of this soft tissue showed pulmonary artery intimal sarcoma and she was commenced on chemotherapy and radiotherapy. The patient unfortunately developed brain metastasis 7 months after initial diagnosis and succumbed to this. We describe the imaging features, diagnosis and management of this uncommon malignancy.

This case highlights the role of imaging in recognising this potentially lethal tumour as it can be easily confused with pulmonary thromboembolic disease and in our case large vessel vasculitis (Hughes-Stovin syndrome).

9. Preclinical Testing Of TRAIL Therapeutics For Sarcoma

Conclusions It suggests that a sarcoma-specific patient reported outcome measure would be useful not just for use by clinicians and patients but also to facilitate development of a rigorous body of research evidence for this population.

8. A rare case of primary intimal sarcoma of the pulmonary artery

Dr. Bindu George, Nottingham University Hospitals NHS Trust; Dr. Cheika Kennedy, Nottingham University Hospitals NHS Trust Pulmonary artery intimal sarcoma is a rare but highly aggressive malignant tumour with a poor prognosis. This can frequently be misdiagnosed on clinical presentation as pulmonary thromboembolism. We present

Zakareya Gamie, Newcastle University; Kenneth Rankin, Newcastle University; Anja Krippner-Heidenreich, Northern Institute for Cancer Research, Newcastle University, Newcastle Upon Tyne, United Kingdom Craig Gerrand, Consultant Orthopaedic Surgeon and Honorary Senior Lecturer, Newcastle University, North of England Bone and Soft Tissue Tumour Service, Freeman Hospital, Newcastle upon Tyne Background Despite intensive, multimodal therapy, patients with bone sarcomas have poor 5-year survival, close to 50%. TNF-related apoptosis-inducing ligand (TRAIL) can induce apoptosis in cancer cells, via the extrinsic pathway, after binding to its death receptors: DR4 and DR5, while sparing nontransformed cells. TRAIL provides

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The aim of this review is to describe the impact of sarcoma on patients psychosocial wellbeing. Specific objectives are to: - Identify published research on patientâ&#x20AC;&#x2122;s psychosocial wellbeing; - Identify interventions that have been developed to improve psychosocial wellbeing; - Critique the methodological quality of identified studies; - Determine factors which influence psychosocial wellbeing; - Make recommendations for future research and clinical practice.

an approach that can overcome current therapeutic limitations, such as drug resistance and toxicity associated with high doses, when administered alone or combined with conventional therapies.

Moreover, resistance of the chondrosarcoma SW1353 cell line to crosslinked forms of TRAIL can be overcome by the addition of the smac mimetic Birinapant, an inhibitor of the Inhibitor of Apoptosis Proteins (IAPs).

Objectives To characterise sarcoma cells for Death Receptor (DR) expression and factors that render them susceptible to TRAIL therapy, and to assess the effectiveness of different forms of TRAIL. Study Design & Methods We characterized osteosarcoma cell lines (U2OS, SAOS-2 and SJSA-1), Ewingâ&#x20AC;&#x2122;s sarcoma cell line (TC-71), bone fibrosarcoma cell line (HT1080), chondrosarcoma cell line (SW1353), for Death Receptor 4 (DR4) and Death Receptor 5 (DR5) expression at the RNA level using RT-PCR, protein level using Western Blotting and at the cell surface using Flow cytometry. Sarcoma cell lines, Normal Human Dermal Fibroblast (NHDF) cells, Bone Marrow-Derived Mesenchymal Stem Cells (BMMSCs) and human hepatocytes (HHL5) were exposed to crosslinked and non-crosslinked forms of TRAIL and effects on cell proliferation and cytotoxicity were assessed using the IncuCyteÂŽ ZOOM Live-Cell Analysis System and WST-8 Cell Proliferation Assay.

Conclusions Our pilot in vitro data indicate that osteosarcoma cell lines such as U2OS can be killed by pre-oligomerised versions of TRAIL and more resistant cell lines such as SJSA-1 can be sensitised to TRAIL by current chemotherapeutics. Furthermore, resistant cell lines such as SW1353 can be sensitised

by Birinapant, which is an improvement on previous smac mimetics and is currently being used in the clinic and in clinical trials for metastatic colorectal cancer, and head and neck cancer. Previous in vivo studies, however, indicate that effective TRAIL concentrations are often not achieved at the site of the tumour. We propose, therefore, to use newly designed scFv:TRAIL variants which are pre-oligomerised and stabilised derivatives of TRAIL, containing a scFvfragment specific for cell surface molecules such as NG2 that we have found to be highly expressed in some bone sarcoma cell lines. This can help improve the selectivity, half-life and potentially reduce cytotoxic effects to normal cells.

Results DR5 were found to be upregulated in sarcoma cell lines at the RNA and protein levels. Crosslinked forms of TRAIL had a more potent effect at reducing cell proliferation and cytotoxicity when compared to non-crosslinked forms of TRAIL. NHDF and BMMSCs were resistant to TRAIL therapy; however, HHL5 cells were sensitive. Our data demonstrates significantly enhanced cell killing of SuperKillerTRAIL (SKT) on osteosarcoma cell lines (U2OS, SJSA-1) when combined with current chemotherapeutics used in bone sarcoma such as doxorubicin (U2OS: IC50=1.7uM, doxorubicin alone vs 0.8uM, doxorubicin with SKT); (SJSA-1: IC50= 8.6uM vs 0.2uM), while the combination is significantly less toxic to a Normal Human Dermal Fibroblast cell line (NHDF) (IC50=0.8uM U2OS vs 1.8uM NHDF).

10. Radiotherapy for the palliation of advanced sarcomas - The effectiveness of radiotherapy in providing symptomatic improvement for advanced sarcomas in a single-centre cohort.

Hannah Tween, Queen Elizabeth Hospital Birmingam; Jenny Sherriff, Queen Elizabeth Hospital Birmingam; David Peake - Queen Elizabeth Hospital Birmingam David Spooner - Queen Elizabeth Hospital Birmingam BACKGROUND Sarcomas are rare and heterogenous tumours with a large proportion of patients requiring palliative intervention. They are regarded as relatively radioresistant and therefore achieving good palliation with radiation may require larger doses than for

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role of a Sarcoma Service Improvement Manager. Everyone needs one?

METHOD Data was retrospectively collected for patients treated with palliative radiotherapy between July 2010 and August 2016 at one institution. Radiotherapy treatment plans and clinic letters were reviewed with data collected including indication, dose fractionation schedule, sarcoma subtype and treatment outcome (clinical and symptomatic response).

RESULTS 86 patients with 105 sites were treated using 22 different dose fractionation schedules.

Median patient age was 59 (range 8-90) years. These included 85 soft tissue and 13 bone sarcomas. Data on symptomatic improvement was available in 57% and 67% respectively. 77% soft tissue and 50% bone sarcoma patients reported symptomatic improvement. Uncontrolled pain was the main indication. No difference in response between different dose fractionation schedules was observed.

CONCLUSION Palliative radiotherapy offers symptomatic improvement for sarcoma patients. These results are limited by the heterogenous study population which includes different sarcoma subtypes with different radiosensitivities, treated with different radiotherapy schedules. Despite this radiotherapy appears to be an effective treatment for symptom control with two thirds of patients reporting symptomatic improvement. Further prospective data collection is needed considering sarcoma subtype radiosensitivity, to determine appropriate palliative dose fractionation schedules.

11. Transforming Sarcoma Services â&#x20AC;&#x201C; the

Gemma French, University College London Hospital; Gemma French, University College London Hospital; Jeremy Whelan - University College London Hospital Julie Woodford - Royal National Orthopaedic Hospital Beatrice Seddon - University College London Hospital Anne McTiernan University College London Hospital Problem The London Sarcoma Service provides comprehensive management of bone and soft tissue sarcomas. Key challenges facing this service include: complex patient pathways within London and South East England, care delivery involving several NHS Trusts, improving patient experiences, managing increasing referrals and ensuring robust governance. A Sarcoma Improvement Manager (SIM) role was developed to respond to these needs.

Achievements A clinical database has been developed by the SIM, improving data collection and outcomes analysis; allowing benchmarking against other sarcoma centres. The SIM has coordinated the implementation of a comprehensive Service Improvement Programme following a successful away day, encouraging clinical engagement in systems change and maximising innovation in service delivery. The SIM supports the London and South East Sarcoma Network Sarcoma Advisory Group and has introduced an effective communication directory of Sarcoma Lead Clinicians in 60 Trusts. A gap analysis indicated a disparity of adherence to guidelines, protocols, and quality standards and a lack of awareness of pathways amongst primary and secondary care. Pathways have been formalised and shared alongside a list of designated practitioners. Referral mapping has identified geographical â&#x20AC;&#x2DC;hotspotsâ&#x20AC;&#x2122; which may benefit from the establishment of sarcoma diagnostic clinics. The SIM also leads on peer review, patient involvement and experience, website development and

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more common solid tumour types. Limited data is available regarding appropriate palliative radiotherapy dose fractionation.

This case series aims to assess the effectiveness of radiotherapy in providing symptomatic improvement for advanced sarcomas.

the strategy of the service.

Conclusion Sarcoma services must be responsive to increased activity, rising patient expectations and commissioning changes. A dedicated Improvement Manager is an invaluable resource. If similar posts are created to work across networks, opportunities for shared learning and efficiencies will be realised.

12. 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

Victoria Ingleson, Cambridge University Hospitals NHS Foundation Trust; 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 for further adjuvant imatinib. SSGXVIII study highlighted high mitotic index and nongastric 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 completion of 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 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: Recurrence-free survival (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 = 52.

UK = 7 (target = 20) Centres: 5 open 3 pending UK accrual until March 2019.

13 Pazopanib in Advanced Soft Tissue Sarcoma; Scottish Study Update

Sara Walker, Beatson West Of Scotland Cancer Centre; Jeff White, Beatson West Of Scotland Cancer Centre; Walker SHJ; Fulton BA; White JD; Vishan U; McCleery M; Cowie F; Nixon I; Hayward L; Ferguson M; McGoldrick T, on behalf of the Scottish Sarcoma Network Background: Pazopanib became a treatment option for metastastic soft tissue sarcoma (STS), after failure of standard chemotherapy, following the PALETTE study (2012), which showed improvement in progression-free survival (PFS). In 2015 retrospective analysis of 13 patients with metastatic STS treated with pazopanib was collected; we have now reviewed a further 2 years worth of data throughout the four Sarcoma centres in Scotland. Methods: Retrospective analysis of 31 patients with metastatic STS treated with pazopanib was performed. The primary outcomes

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14. Quantitative analysis of complications following sarcoma surgery with or without radiotherapy. East Midlands Sarcoma Service Experience

Moataz Abdelreheem, East Midlands Sarcoma Service, Nottingham; MS Tamimy, East Midlands Sarcoma Service, Nottingham; DPS Foong, A Raurell, RU Ashford, KH Steele (East Midlands Sarcoma Service) Radiotherapy (RT) is increasingly utilized in treatment of high grade sarcomas in combination with surgery. RT is associated with post-operative complications. To compare the number and types of complications for sarcomas treated with surgery, with and without RT. A five-year retrospective review of patients was done who underwent sarcoma resection with primary closure between 2012 and 2016.

283 patients underwent surgical treatment for extremity sarcoma.135 patients (47.7%) underwent sarcoma resection with direct closure. These patients were divided into three groups: Group A (no RT), Group B (neoadjuvant RT) and Group C (adjuvant RT).

Complications were compared in the three groups. There were 73 patients in group A (54.1%), 26 in group B (19.3%), 36 in group C (26.7%). Complications in each group were A - 24.7% , B - 50% and C - 13.9% . The most common complication in group A and C was seroma (38.9% and 80% of complications respectively). Seromas were seen in 3 out of 13 patients with complications in group B (23.1%). Wound infection was the most common complication in Group B (46.2%), compared with 22.2% in Group A and none in Group C. Wound healing problems were higher among group A and B (27.8% and 23.1%, respectively) than group C (20%).

Neo-adjuvant RT is associated with highest complication rate in wounds that are closed primarily. Consideration should be given to utilize reconstructive techniques to reduce complications.

15. Uterine Leiomyosarcoma. Referral patterns to the Sarcoma Unit at The Royal Marsden Hospital

Edward Nganga, Royal Marsden Hospital; Christina Messiou, Royal Marsden Hospital; D. Kazzaz, J. Eastman, C Benson, A. Miah, E. Moskovic, A. Sohaib.

Background: Uterine leiomyosarcomas(LMS) are rare and aggressive malignancies with a poor prognosis. It accounts for 8% of uterine cancer and 30% of uterine sarcoma.

Diagnosis is often made after surgery for presumed benign disease. There are no defined standards to distinguish between leiomyoma and leiomyosarcoma on imaging.

Purpose: The aim of this project was to evaluate the referral pathway of patients with uterine LMS to our institution.

Materials and Methods: A retrospective study of patients on the

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were PFS; overall survival (OS); toxicity data and tolerability of therapy. Results: Retrospective analysis identified 31 patients in total treated with pazopanib (median age 53.7years). All patients had advanced non-adipocyte, angiogenesis inhibitornaïve STS prior to commencing therapy.

Median PFS was 3.9 months (95% CI 2.2-5.6 months) and median OS 7.2 months (95% CI 4.5-9.6 months). 7 patients (23%) suffered grade 3-4 toxicity: diarrhoea; skin/hair pigmentation; LFT derangement; ‘unknown’.

The most common grade 1-2 adverse events were skin/hair pigmentation (48%), diarrhoea (42%) and hypertension (42%).

Conclusion: This retrospective analysis shows that while PFS is comparable to the influential PALETTE trial, OS is worse (7.2 months vs. 12.5months). We believe our data reveals more ‘real-life’ outcomes and experience, and continues to highlight that Pazopanib is a well-tolerated treatment option in patients with metastatic STS that provides an improvement in PFS. Further review of a subset of the patients to compare RECIST (Response Evaluation Criteria in Solid Tumours) response with clinically defined benefit and time to best RECIST response is ongoing.

RMH sarcoma database registered with uterine LMS was performed (2008-2017).

Data collected: preoperative LDH, staging, suspicion for sarcoma raised by local radiologist, extra-uterine disease on preoperative imaging, discussion at RMH Sarcoma MDT, date and location of surgery and date of referral to RMH. Results: 85 patients (age range 33-73) were identified. 13% had LDH check preoperatively and 36% of these had raised LDH. 41% had a staging scan preoperatively -27% patients had extrauterine disease on preoperative imaging and 14% had metastatic disease. 21% had suspicion for sarcoma documented on preoperative local imaging reports. 7% had surgery at RMH. 75% were referred postoperatively. 16% of patients with imaging suspicion for sarcoma did not have staging preoperatively.

Conclusion: Improved communication pathways within our network are required to reduce the number of patients with suspected uterine LMS and metastatic disease from undergoing surgery unnecessarily. Suspicion for sarcoma on preoperative imaging should trigger staging investigations and specialist referral. With the exception of bowel obstruction radiotherapy can be used rather than surgery to palliate symptoms and bleeding.

16. Secondary angiosarcoma in previously irradiated breast cancer patients - review of a regional experience

Elena Cojocaru, Cambridge University Hospital, Oncology Department; Helen Hatcher, Cambridge University Hospital, Oncology Department; Han Wong, Gail Horan, Sarah Prewett, Dochka Davidson All authors are affiliated to: Addenbrookes Hospital, Cambridge University Hospitals, Oncology Department Background: Secondary angiosarcoma in previously

irradiated breast cancer is very rare. We evaluated treatment and survival in a cohort of patients diagnosed with radiationinduced breast angiosarcoma.

Methods: We performed a retrospective study of patients diagnosed in our regional service.

We analysed breast cancer history and secondary sarcoma treatments and survival.

Results: Twelve patients developed secondary angiosarcoma after receiving breast radiotherapy. Primary histology included DCIS (1 patient), invasive ductal carcinoma (8 patients) and invasive lobular carcinoma (1 patient). All breast tumours were hormone positive. Treatment of breast cancer included wide local excision and adjuvant radiotherapy (12 patients), adjuvant chemotherapy (4 patients) and hormonotherapy (12 patients). Median time to diagnosis of secondary angiosarcoma 6 years [3;12]. Treatment of angiosarcoma: excision (7 patients) or excision and perioperative chemotherapy with liposomal doxorubicin (5 patients). Eight patients (66,6%) had tumour recurrence. Median PFS was 26 months [2;56]. 3 of 5 patients that received perioperative chemotherapy had a very rapid progression (median PFS 3 months). Initial relapse was local and treatment was excision alone for 4 patients, excision and post-operative chemotherapy with paclitaxel (3 patients) and definitive chemotherapy with paclitaxel for one patient. Median PFS with second line-paclitaxel was 9 months [4;16]. Three patients developed distant metastases in brain, lung and base of skull. Median survival calculated was 7 years [3; 7,7].

Conclusion: Treatment for radiation-induced angiosarcoma comprises complete excision.

Perioperative liposomal doxorubicin could be given in aggressive or large tumours to facilitate surgery. Weekly paclitaxel appeared to be associated with improved survival.

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Dr Radha Todd, Northern Centre for Cancer Care, Newcastle

18. Claudin-3 localization in Schwann cell - like- cell and Endothelial cell in Neurofibromas

Background: Myxoid liposarcoma is a specific histological subtype that accounts for 35% of liposarcomas. It is associated with specific chromosomal translocations resulting in the formation of CHOP/FUS and CHOP/ EWS fusion proteins. A high sensitivity of myxoid liposarcoma to trabectedin was been previously reported.

M.H.Sulaiman, Ahmadu Bello University Zaria, Nigeria; Zaria; J Imam - Ahmadu Bello University Zaria, Nigeria; M.N.Ali Ahmadu Bello University Zaria, Nigeria; A. Dodson - School of Translational Medicine, University of Liverpool, UK; T.R. Helliwell School of Translational Medicine, University of Liverpool, UK; C.A.Kudi - University of Plymouth, UK

Case report: We report the case of a 38-year old man diagnosed with right thigh myxoid liposarcoma in December 2011. There was also limited bone and lung metastases at diagnosis. The patient received treatment with neoadjuvant doxorubicin/ cyclophosphamide and radiotherapy. There was good response in the primary tumour and resolution of lung metastases. The primary lesion was resected in August 2011, however progressive lung metastasis was reported in April 2012. This was treated with a wedge resection.

In January 2013, we detected new lung nodules and subsequently commenced treatment with trabectedin 1.5mg/m2 /24-hour infusion.

The initial response was good with stable disease, however there were haematological abnormalities with a delayed full blood count (FBC) recovery at cycle five and cycle six, hence trabectedin dose was reduced by 25%. Due to further delayed FBC recovery, it was decided to change the treatment to a four-weekly cycle regime. The response has led to significant long-term symptomatic improvement and the patient is due to receive cycle forty-six in November.

Introduction: Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with incident of 1 in 2600. .Most of the cases are familial (inherited), while few cases occur as de novo (sporadic) mutations in primarily paternally derived chromosomes. Claudins are tight junction proteins expressed in epithelial and endothelial cells and form paracellular barriers and pores. Some epithelial cancers have increased expression of claudins, while others have decreased expression levels in specific claudins.

Conclusions: This case suggests that trabectedin represent a feasible therapeutic option for patients with myxoid liposarcoma, with meaningful long-term clinical benefits and an acceptable and manageable safety profile.

Aim of the study: The study was carried out in order to immunohistologically demonstrate the immunolocalization of claudin-3 in Schwann cell like-cell and endothelial cells of familial and sporadic neurofibromas.

Methods: Nerve sheath tumour samples were obtained from patients who attended Liverpool and Broadgreen University Hospital Trust. Normal peripheral nerves from amputation stumps served as positive controls. The patient list is made up of 11 females and 10 males with mean age of 53 years. Standard operating procedure of School of Translational Medicine, University of Liverpool was utilized in the immunohistological technique.

Results: The familial and sporadic NF1 exhibited

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17. Prolonged activity of Trabectedin in Myxoid Liposarcoma â&#x20AC;&#x201C; A Case Report

moderate membrane immunopositivity of the endothelial cells with claudin -3.

Additionally, we observed immunostaining of cell nuclei that is suggestive of Schwann cell nuclei in 2/14 (14%) of the familial neurofibromas and in all 7/7 (100%) of the sporadic neurofibromas.

Conclusion: The nuclei staining observed in the sporadic and familial neurofibromas may be due to the NF1 genotype in which the Schwann cells harbours the mutant allele (NF1-/-).

Thus the Schwann cells are presumably the proliferating cell population in these neurofibromas. Therefore, the nuclei localization of claudin- 3 in Schwann cells may be an indicator of proliferating status of the cells.

19. Fibromatosis of the breast – A 10 year institutional experience and review of the literature

Michael R Boland, Dept. of Breast Surgery, St James’ Hospital, Dublin 8, Ireland; Prof Elizabeth Connolly, Dept. of Breast Surgery, St James’ Hospital, Dublin 8, Ireland; J O’Mahony, S O’Keeffe, C Gillham, A Maguire, D Alazawi, TJ Boyle Departments of Breast Surgery, Radiology, Radiation Oncology and Pathology, St James’ Hospital, Dublin 8, Ireland

search of MEDLINE/Pubmed, Embase, and the Cochrane Library was performed. Studies relating to breast fibromatosis and its management were identified and reviewed.

Results Seven patients were identified. Median age at diagnosis was 37(Range: 22-70) and all patients were diagnosed with core biopsy. The most useful imaging modality in diagnosis was ultrasonography (6/7 patients with abnormalities) followed by magnetic resonance imaging. 5/7 were treated surgically whilst 2/7 were treated using a watch and wait approach. 4/5(80%) required re-excision of margins and 1/5(20%) had recurrence after surgery. On review of the literature, there is no dedicated guideline in place for the management of breast fibromatoses. Currently a watch and wait approach is favoured over surgical intervention due to high levels of recurrence and all cases should be discussed at a sarcoma multidisciplinary team meeting. The use of tyrosine kinase inhibitors should also be considered.

Conclusions Breast fibromatosis is rare but affects young patients and often requires further surgery.

Dedicated guidelines are required to ensure best outcomes.

20. A Mother’s Grief: New Insights into Osteosarcoma from the Application of Aerospace Engineering Principles to Patient-Derived Genomic and Medical History Data

Background Breast fibromatoses are a rare clinical entity but pose significant diagnostic and therapeutic challenges. The aim was to review our institutional experience of management of breast fibromatoses and provide an up-to-date review of current available literature on such management.

Theresa Beech, NASA; Peter Anderson, Clevelend Clinic; Colleen Pearson - Boston University Medical Centre

Methods A search of pathological databases within our institution for all patients diagnosed with fibromatosis of the breast over a ten year period (2007-2016) was performed.

Clinicopathological characteristics and modes of treatment were recorded for each patient. Concurrently a comprehensive

Relapsed/refractory/metastatic osteosarcoma is an intractable disease with poor outcome. Treatments have not changed in over 30 years and new approaches are urgently needed. Applying analysis approaches common in aerospace engineering to patient/parent derived genomic and medical history data, new

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METHODS: Genomic reports (88% Foundation One, 7% MSKCC IMPACT, 5% other) and medical history information of >65 tumors were provided by patients/parents. Genetic sequencing data on 63 pediatric tumors was downloaded from the Foundation One pediatric data portal. IRB approval for data capture and analysis was granted

by Hummingbird IRB, Cambridge, MA (IRB# 2017-26). Multi-variate regression analysis detected genomic patterns, and trends between tumor genomics and patient outcome. Satellite communications network/traffic analysis principles applied to osteosarcoma cell signaling pathways detected potential weaknesses.

RESULTS: Mean number of genomic anomalies (including Variants of Unknown Significance) is 13.5, range 1 - 46; mean age is 16, range 5 - 52; sex ratio is 61%/39% male/female.

Co-amplification loci occur at 8q21-8q24 (MYC, RAD21, RUNX1T1) and 17p1117p12 (C17orf39, NCOR1, FLCN). ATRX and MYC are mutually exclusive. Alternative Lengthening of Telomeres appears in 4050% of tumors. Multi-variate regression analysis of top 50 genes yields 7 genomic sub-types. Network/traffic analysis detected a potential chokepoint at ROCK1. MYC amplified tumors trend to an explosive disease which is almost always fatal. Initial disease presentation is not significant, nor is % tumor necrosis (mean 54%, range 5-95%).

ATRX inactivated tumors begin to trend to an aggressive disease with poor outcome.

RB1 tumors have a significantly higher mean % necrosis (86%) compared to others (43%).

21. Restructuring the Sarcoma MDT: our experience as a tertiary referral centre

Jennifer Murphy, Royal Orthopaedic Hospital, Birmingham; Rajesh Botchu, Royal Orthopaedic Hospital, Birmingham; Anish Patel, Royal Orthopaedic Hospital,

Birmingham; Steven James, Royal Orthopaedic Hospital, Birmingham; AM Davies, Royal Orthopaedic Hospital, Birmingham Background Since its introduction in the mid 1990â&#x20AC;&#x2122;s, the multidisciplinary team (MDT) has been central to cancer management. In recent years there has been increasing demands on the MDT. As a tertiary referral centre for bone and soft tissue sarcoma, we have introduced a daily Diagnostic MDT meeting (DMDT) in an attempt to reduce the workload at the weekly Sarcoma MDT meeting. We describe our MDT experience since its introduction in 1993 and its evolution since the introduction of a daily DMDT meeting.

Materials and methods The number of cases discussed per MDT since 1993 to present were recorded, including cases for histology review and management discussion. Cases discussed at DMDT since its introduction were also recorded and its impact on the main Sarcoma MDT was assessed.

Results: At its introduction in 1993, the average number of cases discussed at the Sarcoma MDT was 15 per MDT (max of 31 cases).

By 2015, the average number of cases discussed was 74 (max of 104 cases). The daily DMDT was introduced in March 2017 and since its introduction an average of 16 cases are discussed per DMDT. Since the introduction of the DMDT, the average number of cases discussed at the Sarcoma MDT is now 61 (max 74 cases).

Conclusion: The MDT structure must adapt to cope with the increasing demand. The introduction of a daily DMDT in our institution has had a positive impact on the workload of our Sarcoma MDT.

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insights into this aggressive disease can be gained.

22. Neutrophil:Lymphocyte Ratio, Albumin and Platelets In Early Relapse In Soft Tissue Sarcoma

23. Radiology recording of sarcoma MDT outcomes

T Bean, Plymouth Hospitals NHS Trust; P Suresh, Plymouth Hospitals NHS Trust

Robert U Ashford, East Midlands Sarcoma Service; Robert U Ashford, East Midlands Sarcoma Service; Nicholas C. Eastley, East Midlands Sarcoma Service; Thomas A. McCulloch, East Midlands Sarcoma Service A number of inflammatory markers and conventional blood tests have shown promise in predicting prognosis in malignancy. Identifying patients likely to relapse at diagnosis can enable targetted therapy earlier in the cancer pathway.

The objective was to determine whether pre-treatment Neutrophil:Lymphocyte Ratio (NLR), Albumin and Platelets could predict early relapse (less than 2 years) We identified from a sarcoma database patients who relapsed early after planned curative treatment (less than 2 years). Preoperative bloods were reviewed from electronic records and NLR, platelets and albumin were measured. An age, sex and tumour pathology matched control group was identified and the outcomes compared. 31 patients (12 male, 5 female) mean age 59 years (range 18 to 79 years) were identified.

The primary tumour was 13 pleomorphic sarcoma, 7 synovial, 3 leiomyosarcoma, 3 myxofibrosarcoma and 3 other. The majority of relapses were in the lung. The mean time to relapse was 12 months . 18 patients relapsed in the first year, 13 in the second.

Elevated NLRs were seen in 12 of 31 the early relapse group particularly In those that relapsed in the first year (9 of 18 versus 3 of 12). High platelet counts (7 of 31; 4 versus 3) and low albumin levels (6 of 31, 3 versus 3) were also seen in those relapsing early.

Elevated NLR and platelets and low albumin are common in patients with early relapse of STS. A wider prospective study may be useful in determining the relevance of these markers to soft tissue sarcoma.

We present a quality improvement project aimed at improving the reporting of sarcoma multidisciplinary team meeting (MDTM) discussions by our radiology team.

The MDTM report is a document which should summarize the radiology input given during the meeting and can be used to document additional discussions regarding the patientâ&#x20AC;&#x2122;s imaging. The report aids effective MDT working by contributing to continuity of information and care between different providers, and this ultimately improves the quality of patient care.

The Royal College of Radiologists (RCR) guidelines on Cancer MDT team meetings recommend that all images discussed at MDTMs should have a supplementary report identifying the images have been reviewed, the histological diagnosis and the MDTM management plan documented. They also indicate that major differences of opinion or discrepancies should be recorded.

Following audit of the radiologist recording of MDT outcomes against the RCR guidance and regional MDT records, we introduced steps and guidelines aimed at improving and standardising radiology MDT reports.

We are currently re-auditing our radiology MDT records of sarcoma MDT outcomes and will present the findings from the completed audit cycle assessing the quality of radiology sarcoma MDT reporting in line with RCR guidance.

24. Primary abdominal and retroperitoneal sarcomas: changing outcomes over time at a single institution

Max Almond, University Hospital Birmingham; Marco Fiore, Istituto Nazionale dei Tumori, Milan, Italy; James Hodson (Department of Medical Statistics, University of Birmingham) Alessandro Gronchi (Istituto Nazionale dei Tumori, Milan, Italy)

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Patients and methods: Four hundred and ninety-one patients with primary retroperitoneal, abdominal and/ or pelvic soft tissue sarcoma treated at the Istituto Nazionale dei Tumori, Milan, Italy, between 2002 and 2016 were identified from a prospectively maintained Sarcoma database. Patients were categorised into three 4-year groups according to the timing of their ďŹ rst operation. Data was abstracted on age, sex, histology, margin status, surgical morbidity, extent of surgery, use of neoadjuvant/adjuvant strategies, and dates of last follow-up, local and/or distant recurrence and/or death. Statistical analysis is ongoing to calculate overall survival, sarcoma-specific survival, and local and distant metastasis-free survival. The impact of date of surgery with respect to a range of outcomes is being evaluated.

Conclusions: Data analysis will be completed shortly enabling presentation of outcomes from this high-volume unit by the time of the BSG conference.

25. The Role of DMSA Imaging in Surgical Planning for Patients with Retroperitoneal Soft Tissue Sarcoma

Miss HL Tattersall, College of Medical and Dental Sciences, University of Birmingham; Mr SJ Ford, Midlands Abdominal and Retroperitoneal Sarcoma Unit (MARSU), University Hospitals Birmingham Foundation Trust; Mr LM Almond - Midlands Abdominal and Retroperitoneal Sarcoma Unit (MARSU), University Hospitals Birmingham Foundation Trust Mr A Desai - Midlands Abdominal and Retroperitoneal Sarcoma Unit (MARSU), University Hospitals Birmingham Foundation Trust Professor D Gourevitch - Midlands Abdominal and Retroperitoneal Sarcoma Unit (MARSU), University Hospitals

Birmingham Foundation Trust INTRODUCTION The standard of care for the management of primary retroperitoneal sarcoma (RPS) is multi-visceral resection. This approach maximises local disease control at the expense of ipsilateral kidney resection.

All patients undergoing surgery for RPS at University Hospitals Birmingham (UHB) undergo extensive pre-operative investigation to assist in surgical planning.

Assessment includes measurement of split renal function with DMSA imaging.

PURPOSE To evaluate the influence of DMSA imaging on surgical planning in patients undergoing excision of RPS. METHODS Retrospective data collection and analysis of sequential patients referred to The Sarcoma Unit, UHB between April 2008 and September 2017 with RPS. A total of 117 were subject to DMSA imaging. The results of DMSA imaging were considered in relation to other pre-operative imaging and biochemical renal function.

RESULTS All 117 patients were included. DMSA imaging showed 53 of 117 (45.3%) patients had an uneven split renal function with one side contributing less than 45%.

Disproportionately less function was correlated with RPS laterality in 39 of 53 (73.6%). Of these 39 patients, 21 (53.8%) had prior features suggestive of potential unilateral renal impairment on preoperative CT imaging. DMSA imaging had no correlation with pre-operative renal function. There were 2 (2.1%) examples of an unexpected DMSA imaging outcome that influenced surgical planning necessitating renal preservation on the pathological side.

CONCLUSION Pre-operative DMSA imaging in RPS offers some reassurance although directly alters management in less than 3% of cases. DMSA imaging should not be routinely utilised

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Background: To assess changes in outcomes over time in patients with abdominal or retroperitoneal sarcoma treated at a high-volume European centre.

in the pre-operative assessment of RPS patients.

26. Is thalidomide of use in angiosarcoma - what we can learn from dogs

Jonathan Bray, Fitzpatrick Referrals Oncology and Soft Tissue; Jonathan Bray, Fitzpatrick Referrals Oncology and Soft Tissue; Nick Cave, Massey University; John Munday, Massey University; Geoff Orbell, IDEXX laboratories Introduction: Haemangiosarcoma (HSA) is a malignant neoplasm of the dog that develops in the spleen, heart and liver. Survival times after resection of the primary tumour are poor with most dogs dying due to progressive metastatic disease within 2-3 months.

There are close similarities between the gene expression profiles of HSA and human angiosarcoma. This presentation will describe the outcome of this study and discuss how the canine disease may prove a useful research model.

Methods: Fifteen dogs with splenic haemangiosarcoma were included.

Following recovery from splenectomy, all dogs received thalidomide continuously until their death. Tumours were staged using computed tomographic scans of the chest and abdomen immediately prior to starting thalidomide treatment and again 3 months later. Survival times were compared to a contemporaneous group of 16 dogs that did not receive any chemotherapeutic agents after surgery Results: The median survival time (MST) of dogs receiving thalidomide was 172 days (95% CI 93-250 days). This was significantly longer than the contemporaneous group (MST 32 days (p=0.001). Dogs with stage 2 disease that received thalidomide had a longer MST than dogs with stage 3 disease (MST 303 vs 40 days).

Conclusion: Treatment using thalidomide appears to improve survival of dogs with splenic haemangiosarcoma. Thalidomide targets not only angiogenesis, but also the immunomodulatory and pro-inflammatory components considered important for establishment and progression of metastatic nodules. The canine disease may provide a comparative model of spontaneous disease to allow investigation of the microenvironment on tumour progression and thalidomideâ&#x20AC;&#x2122;s role in influencing oncogenesis.

27. Retroperitoneal sarcomas; MDT burden and diagnostic service

Olivier Dick, NUH; Cheika Kennedy, NUH; Alastair Simpson, RP Surgeon; Tom McCulloch, Sarcoma pathologist Referrals to the East Midlands Retroperitoneal Sarcoma Service have doubled since 2014, averaging 50 discussions per month. Our population serves 4.5 million and as such we should see no more than 20 new RP sarcoma diagnoses per year. However, the burden of MDT review has led us to question the appropriateness of referrals to our service.

A retrospective review of those lesions highlights key radiological findings that may alleviate MDT burden. However, our RP biopsy service to the region routinely yields 37 - 40% non-sarcomatous diagnoses even after MDT review and raises the question; is the RP sarcoma service as much a diagnostic service as a sarcoma service?

28. Mechanisms Of Failure And Long Term Survival Of Total Femur Replacements

Manuel Ricardo Medellin Rincon, Royal Orthopaedic Hospital; Lee Jeys, Royal Orthopaedic Hospital; Tomohiro Fujiwara - Royal Orthopaedic Hospital; Rhys Clark Royal Orthopaedic Hospital AIM:

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MATERIALS AND METHODS: 81 patients treated with total femur prosthesis from 1976 to 2017 were retrospectively evaluated. Implant characteristics, complications and functional results were recorded. A bivariate analysis followed by survival analysis was performed.

RESULTS: The revision free survival of the implant was 71% at 5 years, 63.3% at 10 years and 33.6% at 20 years with a mean MSTS score of 26 (23 â&#x20AC;&#x201C; 28). The mechanisms of failure were infection in 18% (type 4), structural failures in 6% (type 3), tumoral progression in 5% (type 5), aseptic loosening in 2% (type 2) and soft tissue failures in 1% (type 1).

Primary implants had lower infection rates than revision implants (8% vs 25%), (P = 0.001). The rates of infection in silver coated and non-silver coated prosthesis were similar (17,4% vs 19.%) (P = 0.869). 10 % of the patients presented dislocation. Rotating hinge prosthesis had less failures than fixed hinge prosthesis (5,3% vs 11%). The malalignment of the implant was associated with failures. On Cox regression the only independent factor associated with failures was the history of previous surgeries (HR 3,804 - P = 0,043).

CONCLUSIONS: total femur replacement is a reliable reconstruction, knee rotating hinge and dual mobility cups may decrease the risk mechanical and soft tissue failures. The anatomical position of the knee should be restored. Infection remain the main concern, and there is not enough evidence to support the routinely use of silver coated prosthesis.

29. Lengthening Total Femur Arthroplasty, Comparison Between Non-Invasive And Minimally Invasive Prosthesis

Manuel Ricardo Medellin Rincon, Royal Orthopaedic Hospital; Lee Jeys, Royal

AIM: To describe and analyse the survival and complications of non-invasive and minimally invasive lengthening total femur prosthesis.

MATERIALS AND METHODS: 24 expandable total femurs were implanted in 20 patients from 1991 to 2016. Implant characteristics, complications and functional results were recorded. A survival analysis was performed, followed by the classification of failures according to the Modified Henderson System.

RESULTS: The overall survival rates of minimally invasive and noninvasive expandable prosthesis were 78% and 77% at 5 years, 49% and 64% at 10 years and 24% at 20 years in minimally invasive prosthesis. At last follow up 10 implants did not require further surgery. The overall complication rate was 58%. Complications were slightly more common in minimally invasive prosthesis compared to non-invasive ones (64% vs 57%). The mean revision free survival times for minimally invasive and non-invasive prosthesis were 59 months and 49 months respectively. No differences were observed in the overall implant survival, revision free survival or the distribution of complications between the two types of implant. At last follow up, comparing noninvasive and minimally invasive implants, the last ones presented higher rates of leg length discrepancy (36% vs 23%), lower range of movement in the knee joint , and lower MSTS scores (24,7 vs 27) CONCLUSIONS: The survival and complications of noninvasive and minimally invasive lengthening total femurs are comparable; however, patients with non-invasive prosthesis have a more accurate limb length discrepancy correction, a better knee range of movement and an improved overall function.

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Orthopaedic Hospital; Tomohiro Fujiwara - Royal Orthopaedic Hospital Rhys Clark Royal Orthopaedic Hospital

To evaluate the prosthesis survival of total femur prosthesis.

30. Prognostic Factors In The Management Of Giant Cell Tumours Of Extremity Bones With Pathological Fracture

Manuel Ricardo Medellin Rincon, Royal Orthopaedic Hospital; Adesegun Abudu, Royal Orthopaedic Hospital; Tomohiro Fujiwara - Royal Orthopaedic Hospital Jonathan Stevenson - Royal Orthopaedic Hospital Jonathan Gregory - Royal Orthopaedic Hospital Michael Parry - Royal Orthopaedic Hospital Lee Jeys - Michael Parry - Royal Orthopaedic Hospital Roger Tillman - Michael Parry - Royal Orthopaedic Hospital AIM: To investigate the prognostic factors for local recurrence in patients with pathological fracture through Giant Cell tumors of Bone (GCTB).

analysis, the type of surgical treatment performed was the only independent factor predicting local recurrence. All patients treated with Denosumab and intralesional curettage developed local recurrence.

Repeat Surgery was required 30% more after intralesional curettage in comparison with resections.

CONCLUSIONS: En-bloc resection offers lower risks of local recurrence in patients with pathological fractures through Campanacci stage three tumors whilst curettage remains a good option for those with Campanacci stage two tumors. The benefits of Denosumab still remain unclear.

31. Diagnostic accuracy of image-guided needle biopsy for Ewing sarcoma of bone: a 10-year review

MATERIALS AND METHODS: 109 patients presenting with fractures through GCTB treated at our institution between 1995 and 2016 were retrospectively studied. A bivariate analysis was performed, followed by multivariate analysis.

RESULTS: The initial treatment was conservative curettage +/- adjuvants in 61 patients (56%) and en-bloc resection with or without reconstruction in 48 patients (44%). The choice of treatment depended on tumour location, Campanacci tumour staging, intra- articular involvement and fracture displacement. Neoadjuvant Denosumab was used in 9 patients with aggressive fractured Campanacci stage 3 tumors. Local recurrence occurred in 28 patients (25%).

Surgery &gt; 6 weeks following fracture did not affect the risk of recurrence in both surgical groups. In Campanacci stage three tumors, en-bloc resection had lower local recurrences (11%), compared with curettage (46%). In tumors classified as Campanacci 2, intralesional curettage and en bloc resections had similar recurrence rates (21% and 24%, respectively). After multivariate

Sarah Kalus, Royal National Orthopaedic Hospital; Asif Saifuddin, Royal National Orthopaedic Hospital Aim: To determine the diagnostic accuracy of image-guided needle biopsy in 140 cases of Ewing sarcoma diagnosed at the Royal National Orthopaedic Hospital.

Methods: All patients with a histological diagnosis of Ewing sarcoma who underwent imageguided needle biopsy at the Royal National Orthopaedic Hospital between January 2007 and December 2016 were included in this retrospective study. Analysis included mean age, skeletal distribution of Ewing sarcoma, imaging modality used for biopsy guidance, type of anaesthesia, needle type, number of passes, and the type of tissue sampled.

Results: 140 patients were diagnosed with Ewing sarcoma, having an age range of 2-56 years, a mean age of 18.6 years and a M:F ratio of 2:1. 99 patients underwent CT-guided biopsy, of which 75 were performed under general anaesthesia. 39 patients underwent ultrasound-guided biopsy, of which 27

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Discussion This is the first reported case of desmoid fibromatosis secondary to an an ACL reconstruction using an Endobutton.

33. Ten Years Egyptian Experience In Treating Bone Sarcomas

Conclusion: Image-guided needle biopsy can be performed safely and with high accuracy to establish the diagnosis of Ewing sarcoma.

Ehab Saad, Department of clinical oncology, Cairo University, Egypt; Ehab Saad, Department of clinical oncology, Cairo University, Egypt; Ashraf Mounir Department of clinical oncology, Cairo University, Egypt

32. Desmoid Fibromatosis Secondary To ACL Reconstruction With An Endobutton

Introduction and Objectives: Primary bone cancers are rare accounting for about 0.2% of all cancers. Bone sarcomas have three distinct categories: osteosarcoma, Ewing sarcoma, and chondrosarcoma. This work aims at studying the different factors affecting survival for cases of bone sarcoma.

R. Aujla, Nicholas Eastley, Robert U Ashford, Damien McDermott, Thomas A McCulloch East Midlands Sarcoma Service Background Desmoid fibromatosis is a WHO intermediate condition. It is reported following surgery and associated with breast implant surgery. It mimics soft tissue sarcomas and can present as a soft tissue mass. Desmoid fibromatosis has undergone a major management change in recent years with a change from surgical to non-surgical management usually after a period of active observation.

Case Presentation A 29 year old gentleman presented to our sarcoma service with a lump in the lateral aspect of his knee. 18 months previously he had undergone an arthroscopic Anterior Cruciate Ligament reconstruction using a hamstring graft secured with an endobutton on the femoral side. MRI scan revealed a solid mass. Percutaneously biopsy under ultrasound control confirmed the histology to be desmoid fibromatosis.

Outcomes Treatment following diagnosis is by active observation the standard first line management for desmoid fibromatosis as per European guidelines.

Material and Methods: A retrospective study, evaluating 101 patients with bone sarcoma in department of clinical oncology, Cairo University, between January 2005 and January 2016, regarding clinical, epidemiological, pathological characteristics and treatment modalities and their relation to the patients’ survival.

Results: 61/101 of patients were presented with osteosarcoma, 20/101 with Ewing Sarcoma, and 20/101 with chondrosarcoma. For osteosarcoma, significant factors for better survival were necrosis >90 % (p=0.041) on DFS, age < 18 on OS (P=.036), and DFS (p=.039), and absence of metastasis at presentation on OS (p=0.023), and PFS (p=0.019). For Ewing’s sarcoma, significant factors for better survival were the low LDH level on OS (p=0.032) and absence of metastatic disease at presentation on PFS (p=0.048). For chondrosarcoma, no significant factors affected patients’ survival, due to the low number of cases.

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involved general anaesthesia. In 2 patients, the biopsy was performed using both CT and ultrasound guidance, 1 of which was under general anaesthesia. 137 biopsies (97.9%) were diagnostic. Three biopsies performed under CT guidance and general anaesthesia were non-diagnostic, of which a second image-guided needle biopsy was diagnostic in 2 cases.

Conclusions: Ten year analysis of bone sarcoma showed that it is a rare disease comprising only 0.29% of all cancer patients. The patients’ survival is affected by clinical, epidemiological, pathological factors and by the use of different treatment modalities; however, other factors did not meet the statistical significance due to relative small patients’ number.

with adjacent viscera, 30 (38%) requiring ≥2 resected viscera. The median diameter was 23cm and 44 were Grade ≥2 (54%).

Macroscopically clear resection margins were achieved in 68 patients (85%). The 30-day serious morbidity (Clavien-Dindo ≥3) and mortality were 15% (n=12) and 0% respectively. The median length of followup was 22 months (range 2-125). The locoregional recurrence was 27% (n=21) and 75% (n=60) were alive.

34. Single Centre Outcomes from Retroperitoneal Sarcoma Surgery

Conclusion: The majority of patients in this series required adjacent visceral resections and achieved a high rate of complete macroscopic clearance. This approach in a specialist RPS unit was also associated with a low serious complication rate.

James Bunce, Nottingham University Hospitals NHS Trust; Mr J A Simpson, Nottingham University Hospitals NHS Trust; Oliver Peacock, Wayne Croves, Tom McCulloch, Gurminder Mann, David J Humes, Catherine J Walter Introduction: Retroperitoneal sarcomas (RPS) are rare mesenchymal tumours. Specialist centres now advocate a radical approach to the surgical management of RPS, demonstrating improved oncological outcomes. However, there is limited evidence regarding the safety of radical resections and validated oncological outcomes.

The aim of this study is to investigate this specialist practice in a contemporary series in a single UK centre.

Methods: Retrospective data on consecutive patients undergoing RPS surgery at a single tertiary UK institution between April 2006 and October 2017 were collected.

Patient demographics, operative details, histological characteristics, short and longterm outcomes were evaluated, focusing particularly on surgical adverse events and local recurrence.

Results: Eighty patients were included [median age 65, range 22-86; 47 males (59%)]. Sixty-four patients had a primary tumour. Sixty-three patient’s (79%) tumour was resected en-bloc

35. Evaluating the Cost of a Retroperitoneal Sarcoma Service at a Single Centre

James Bunce, Nottingham University Hospitals NHS Trust; Mr J A Simpson, Nottingham University Hospitals NHS Trust; Oliver Peacock, Wayne Croves, Gurminder Mann, Tom McCulloch, David J Humes, Catherine J Walter Background: Retroperitoneal sarcomas (RPS) often present late and have a high tumour burden.

A radical surgical approach is advocated to minimise marginality and decrease the risk of local recurrence. The aim of this study is to review the financial viability of this specialist service at a single UK referral centre.

Methods: Prospective data on consecutive patients undergoing surgery for retroperitoneal tumours at a single tertiary UK institution between October 2016 and September 2017 were collected. Patient demographics, operative details, histological characteristics and short-term outcomes were evaluated, particularly focusing on the Patient Level Information and Costing Systems (PLICS)

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Results: Twenty-five patients were included [median age 66, range 30-86; 15 males (60%)].

Seventeen patients (68%) had histologically confirmed RPS, the remaining 8 were nonsarcomatous lesions. The median length of hospital stay was 8 days (range 2-31 days).

The serious 30-day morbidity and mortality were 8% and 0% respectively. The median operative time was similar between the RPS group (240 mins, range 110-480) and the non-sarcomatous lesions group (255 mins, range 70-390) and a similar frequency of required multi-specialty input (50% versus 53%). The mean income per patient for the RPS group was £18,843 versus a mean cost of £17,520. The mean income per patient for the non-RPS group was £9,127 versus a mean cost of £19,322.

Conclusion: One-third of cases were non-sarcomatous lesions, but required the specific skillset of the RPS team. Tariffs taken directly from the histological diagnosis do not reflect the resource allocation and inadvertently financially penalise the RPS service.

Our experience has been that PR plays a valuable role in palliating bone pain in paediatric osteosarcoma. Prospective evaluation with patient/caregiver-reported outcomes and analgesic use could further validate this treatment option.

36. Is radiation underutilised for palliation in paediatric osteosarcoma?

Neil Wallace, Radiotherapy Department, Cork University Hospital; Paul J Kelly, Radiotherapy Department, Cork University Hospital; Patricia Sheehan, Palliative Care Department, Cork University Hospital Deirdre O’Mahony, Medical Oncology Department, Cork University Hospital

37. Long term use of Trabectadin – 3 cases showing efficacy and tolerability

Dr Lesley Speed, University Hospitals of Leicester NHS Trust; Dr Shiv Uppal, University Hospitals of Leicester NHS Trust

Palliative radiotherapy(PR) is frequently used in the symptomatic management of painful osseous metastases from various underlying primary cancers. Radiation can also be applied to primary tumours of bone – here we discuss our institutional experience of radiotherapy to paediatric patients with osteosarcoma treated with palliative intent.

Four children (ages 9, 10, 16, and 16)

with osteosarcoma were treated with PR over the past five years. All patients were referred from the national paediatric oncology referral centre in a palliative phase of the their illness for palliation of bone pain. One received upfront PR to an inoperable pelvic osteosarcoma after MDT discussion. The others were treated for painful metastases after progressing through multiple lines of treatment with surgery and/or chemotherapy. To date, 4 children have received PR to a total of 15 sites throughout the axial and appendicular skeleton. Dosing and fractionation have been in keeping with typical doses given to adult patients with osseous malignancies in the same anatomical regions. Of the 15 sites treated, 3 sites were re-treated subsequently following a recurrence of local symptoms after an analgesic response to an initial course of PR. Records from clinical consultations and parent and child feedback suggest the overall impact on the children’s pain was above expectation, although formal records of response are limited by the retrospective nature of this review.

Trabectadin now has a well-established role in the management of soft tissue sarcoma.

Ongoing studies are looking at its use as maintenance post first line chemotherapy but there are increasing patient numbers receiving this drug on a long term basis as second or third line treatment. We present 3 cases of patients with metastatic soft tissue sarcoma who have received Trabectadin for over 12 months.

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data.

Case 1 has received 31 cycles over a period of 25 months for metastatic leiomyosarcoma, remains on full dose but now at 4 weekly intervals given the longevity of treatment to allow quality of life. Grade 1 toxicities only reported. This patient had response in disease followed by stability.

Case 2 has received 16 cycles over 16 months for metastatic liposarcoma. Their dose has been reduced to 1.2mg/m2 due to grade 3 neutropenia and thrombocytopenia and subsequently moved to 4 weekly dosing but this is now tolerated well with overall stable disease.

Case 3 received 15 cycles over 14 months, at full dose but on 4 weekly schedule for patient preference. They achieved stable disease of metastatic leiomyosarcoma and then slow progression so stopped their chemotherapy but only ever experienced grade 1 toxicities.

These cases demonstrate patient acceptability and treatment effectiveness over prolonged time periods with this drug.

defined, soft/firm feeling swelling (10x8cm).

On the surface of the swelling, there is a purplish skin mark measuring 3x4cm, the patient reported that the skin mark has changed its shape following the appearance of the swelling. MRI scan reported as haemangioma. The decision was to repeat MRI in 6 months. Unfortunately the patient DNA’d the follow-up clinics (x4) and was discharged. Three years later he was referred to us again, new MRI scan showed interval increase of the swelling, but the radiologist reported as dermatitis on the pre-existing haemangioma. The patient then had biopsy, which confirmed DT. The staging CT is clear.

The patient underwent local wide excision + skin graft in 2013. Recent follow-up in October 2017 show no local recurrence, no metastatic disease.

Conclusion: DT is a very rare, low-grade malignant tumour. It behaviours like a superficial, slowgrowing and ill-defined swelling. MRI could be easily confused with benign superficial vascular lesions. We strongly recommend biopsy if MRI shows any atypical features or enlarging of any vascular lesions.

39. The value of a telephone virtual clinic for a regional sarcoma service

38. Dąbska Tumour --- A Rare Case Report in Adult and Literature Review

Qi Yin, Royal Liverpool University Hospital; Professor Tim Helliwell Department of Pathology Royal Liverpool University Hospital The Dąbska tumour (DT) is a rare low-grade angiosarcoma characterized by papillary endovascular proliferations of atypical endothelial cells and anastomosing vascular channels within the dermis. Since its original description, only 12 cases were reported in adults. We present a very rare DT in adult and discuss the images and 4-year followup result after surgery. A 58-year male, presented with painless, slow-growing swelling over the posterior/medial aspect of left thigh for 9 months with no injury.

Clinically, there is a superficial, raised, ill-

Anita Pabla, East Midlands Sarcoma Service; Robert U Ashford, East Midlands Sarcoma Service / University of Leicester; Lesley Speed [1] Nicholas C Eastley [1,2] Laura Poyntz [2] Joe A Reynolds [2] [1] East Midlands Sarcoma Service, Leicester Royal Infirmary [2] University of Leicester Virtual follow up clinics are increasingly used in the management of fractures and chronic medical conditions. Sarcoma services are delivered regionally and as such patients have to travel long distances and take time off work. In our sarcoma clinics, attendance can often last approximately 2 hours. At Leicester, we introduced a telephone virtual sarcoma clinic in 2017.

We describe the first 9 months of the service 103 patients had virtual (telephone)

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There were no complaints or concerns raised by the patients regarding the virtual clinic. Patients appreciate the avoidance of attendance at regional clinics where things can safely be managed from afar. We do not break bad news over the telephone. Virtual clinics are funded in the NHS at £25 per consultation and with appropriate utilisation can improve the patient experience. Based on our experience we are expanding our virtual clinics.

40. Diagnostic accuracy of CT-guided percutaneous bone biopsy in a tertiary musculoskeletal oncology centre Pedone Luigi, The Royal National Orthopaedic Hospital - NHS Trust; Asif Saiffudin, The Royal National Orthopaedic Hospital - NHS Trust

Results Of the 192 lesions, 6 were excluded as it was impossible to establish a definitive

Conclusion CT guided percutaneous biopsy under GA is a safe and accurate procedure with an excellent success rate for guiding clinical management.

41. The effects of metabolic modulation and immunogenicity in Osteosarcoma

Purpose To assess the diagnostic accuracy of CT-guided percutaneous biopsy of bone lesions referred to a tertiary musculoskeletal oncology centre.

Materials and methods A retrospective review of 192 consecutive CT-guided percutaneous bone biopsies performed in 188 patients, under General Anaesthesia (GA) by an experienced operator (more than 23 years) between October 2016 and October 2017.

diagnosis. Therefore, the study group consisted of 186 lesions in 182 patients.

There were 86 males (47.2%) and 96 females (52.8%), with a mean age of 38 years (range 1 to 88 years). The sites of biopsy were the upper body in 22 (11.7%), the pelvis in 54 (28.8%), the upper limb in 28 (14.9%) and the lower limb in 84 (44.6%). 180 of the 186 biopsies yielded a diagnostic sample (96.77 % overall accuracy) while 6 biopsies were false negative.

Of the latter, the biopsy results showed blood clot in 5 cases and a necrotic infiltrate in one. Further investigation of the 5 cases of blood clot (by repeat biopsy or curettage) showed in 2 ABCs, 1 sarcoid, 1 atypical haemangioma and 1 chondroblastoma with extensive ABC change. For the child with necrotic infiltrate, a bone marrow biopsy was performed and leukaemia was diagnosed. No immediate post-biopsy complications were recorded.

Kheshwant Gill, Cancer Research at University College Cork; Patrick Forde, Cancer Research at University College Cork; Philana Fernandes - Cancer Research at University College Cork Declan Soden Cancer Research at University College Cork Osteosarcoma is the most common (75%) high-grade malignant tumour of the skeletal system in paediatric patients. In 2014, there were 579 new cases of osteosarcoma in the United Kingdom. The mortality rates in osteosarcoma are due to both intrinsic and acquired resistance (60%) to currently used chemotherapies that lead to multidrug resistant phenotypes and the occurrence of ‘second malignancies’. Attempts to improve therapy efficacy by dose escalation, alterations in combinations of chemotherapy

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consultations through the clinic, 47 male 56 female, mean age years (range 21 – 89). 77 patients were of working age. There were 45 patients who were deemed by the sarcoma MDT to go direct to imaging (either crosssectional imaging or ultrasound guided biopsy) and 58 follow up patients. The mean mileage (return journey) saved was 42 (range 3.6 to 176) miles. The patients also avoided car parking costs (£2.80 for 2 hours) or the inconvenience associated with hospital transport. Patients also did not need to take time off work.

and irradiation therapy have not improved survival outcomes.

The tumour microenvironment poses as a major barrier to treatments due to immunosupression by chronic inflammation, tumour survival and angiogenesis. A low presence of immune cells within the tumour microenvionment correlates with a poorer prognosis in cancer. Electroporation is a non-thermal, cell permeabilising technology that renders the treated cell membranes permeable to poorly permeant anti-cancer drugs thus facilitating a potent local cytotoxic effect from the improved cell membrane porosity. Metabolic modulators in combination with chemotherapy has been shown to be effective in cancer treatment, due to increase sensitivity of cancer cells from depletion of intracellular ATP levels from glycolytic modulation. The aims of this study were to examine the cytotoxic effects of electroporation delivered metabolic modulators and low dose chemotherapy that subvert therapy resistance by increasing osteosarcoma sensitivity to existing treatments. Recruiting an immune response by modulating the tumour microenvironment â&#x20AC;&#x201C; cell cycle modulation combined with low-dose chemotherapy, promotes an anti-tumour response and thus improving treatment efficacy and outcome.

42. Mapping the Site of Local Recurrence in Retroperitoneal Liposarcoma (RPLPS)

Sandro Pasquali, Istituto Nazionale dei Tumori, Milan; Sandro Pasquali, Istituto Nazionale dei Tumori, Milan; Max Almond, Hannah Tattersall, Samuel Ford, Anant Desai, Alessandro Gronchi PURPOSE To investigate the occurrence and pattern of local recurrence (LR) using standardised retroperitoneal mapping, in patients with primary RPLPS who underwent extended resection.

METHODS Patients who underwent surgery for

primary RPLPS between 2009 and 2014 in two European centres: Istituto dei Tumori, Milan; and Midlands Abdominal and Retroperitoneal Sarcoma Unit, Birmingham, were identified from the Sarcoma databases at each centre. Patterns of LR were recorded using standardised retroperitoneal mapping based on cross-sectional imaging appearances at first recurrence. Post-LR survival was analysed.

RESULTS Results are currently available only for the Milan cohort, although the authors expect the combined dataset to be available by the time of BSG. 129 patients underwent extended resection for a primary RPLPS of whom 51 (39.5%) developed recurrence after a median follow-up of 44 months.

First treatment failure was by LR, DM or synchronous LR and DM, in 37 (72.5%), 7 (13.7%) and 7 (13.7%) patients respectively.

Retroperitoneal mapping demonstrated that primary and recurrent RPLPS involved a median of 5.5 and 1 station respectively with 82% of recurrent tumours limited to a single station. Multifocal LR was detected in 13 (25.5%) patients. Overall 3-yr postLR survival was 0.34 with worse outcomes in high-grade dedifferentiated tumours.

Patients who underwent further surgery for LR (n=20, p=0.019), those with lower tumour grade (p=0.001) and those with LR within the same stations as the primary (p=0.003) had longer post-LR survival.

CONCLUSIONS In the event of LR in RPLPS, tumours recurred in a single anatomical compartment in 82%. Half of these patients underwent further surgery which correlated with improved survival.

43. Anthracycline-related cardiotoxicity in Ewingâ&#x20AC;&#x2122;s and Osteosarcoma patients

N Osifodunrin, Royal Hospital for Children, Glasgow.; J Sastry, Royal Hospital for Children Glasgow; D Murphy- Royal Hospital for Children, Glasgow M Ronghe- Royal Hospital for Children, Glasgow F Cowie- The Page 68

Background Anthracyclines are included in the multimodal chemotherapy regimens described in the current protocols used across the UK and Europe: Euro Ewings 2012 and EURAMOS-1 for the treatment of Ewing’s sarcoma and osteosarcoma. Although anthracyclines have been shown to improve the outcome of bone sarcomas1 they are also associated with cardiotoxicity.

Cardiovascular complications are the leading non-oncological related causes of morbidity and mortality in childhood cancer survivors 2,3. Certain risk factors associated with cardiotoxicity have been found including higher cumulative anthracycline dose, female sex, younger age at diagnosis and pre-existing cardiovascular disease 4.

Method A retrospective review of all patients <18yrs diagnosed with Ewing’s Sarcoma and osteosarcoma between 2012-2017 was undertaken. Echocardiograms performed pre-treatment, during treatment and after treatment were reviewed. Cardiotoxicity was graded using Common Terminology Criteria for Adverse Events v 3.0.

Results 23 patients were identified, 5 with osteosarcoma and 18 with Ewing’s sarcoma.

2 patients that died during treatment were excluded.11 were female and 10 were male. The median cumulative dose of anthracycline was 380mg/m2. The median age at diagnosis was 11.5 years. There were 2/21 patients with ejection fraction (EF) <50% during treatment (CTCAE Grade 2).

The remaining 19 patients had an EF >60% during and post-treatment. The range of percentage decline in EF was 5-11% with an average of 7.7%. No patients required a dose reduction of anthracycline or treatment for cardiotoxicity.

Conclusion In this small cohort, no patients treated with

anthracyclines as per EURAMOS and EuroEwings protocols had significant problems with cardiotoxicity. Long term follow up however, is necessary to pick up late toxicity.

1. ESMO/ European Sarcoma Network Working Group. Bone sarcomas: ESMO clinical practice guidelined for diagnosis, treatment and follow up. Ann Oncol 2014;25 (Suppl 3): iii113-iii123 2. Mertens AC, Liu Q, Neglia JP et al. Cause- specific late mortality among 5 year survivors of childhood cancer: The Childhood Cancer Survivor Study. J Natl Cancer Inst 2008;100:1368-1379 3.

Tukenova M, Guibout C, Oberlin O et al.

Role of cancer treatment in long-term overall survival and cardiovascular mortality after childhood cancer. J Clin Oncol 2010;28: 1308-1315 4. Lipshultz SE, Alvarez JA. Scully RE. Anthracycline associated cardiotoxicity in survivors of childhood cancer. Heart 2008;94: 525-533

44. A Prognostic Hypoxia Related Gene Signature for Soft Tissue Sarcoma

Laura Forker, University of Manchester; Catharine West, University of Manchester; Lingjian Yang, Joely Irlam, Ananya Choudhury University of Manchester Background Despite high metastasis rates, there is no clear benefit from conventional adjuvant/ neo-adjuvant chemotherapy for localised soft tissue sarcoma (STS). An alternative approach would be to tailor therapy to adverse features of tumour biology present across multiple histologic subtypes. Tumour hypoxia is associated with metastasis in STS and is targetable. This study aimed to derive and validate a hypoxia mRNA signature for STS for biomarker-driven trials of hypoxiatargeted therapy.

Methods Seed genes induced under hypoxia in seven STS cell lines were identified by RNA sequencing. Publically available datasets with whole transcriptome gene expression data and clinical outcome data were

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Beatson Hospital, Glasgow R Jones- The Beatson Hospital, Glasgow R Duncan- Royal Hospital for Children, Glasgow

identified. Primary tumours in a training cohort (French Sarcoma Group) were clustered into two phenotypes by seed gene expression and a hypoxia signature was derived. Prognostic significance of the de novo signature was evaluated in the training and two independent validation cohorts (French Sarcoma Group and the Cancer Genome Atlas).

Results 37 genes were up-regulated by hypoxia in all seven cell lines, and a 24-gene signature was derived. The high-hypoxia phenotype defined by the signature was enriched for well-established hypoxia genes reported in the literature. The signature was prognostic in univariable analysis, and in multivariable analysis in the training (n=183, HR 2.16, P=0.0054) and two independent validation (n=127, HR 3.06, P=0.0019; n=258, HR 2.05, P=0.0098) cohorts. Combining information from the hypoxia signature and a genome instability signature (CINSARC) improved prognostication. Conclusion A 24-gene STS-specific hypoxia signature may be useful for prognostication and identifying patients for hypoxia-targeted therapy in clinical trials.

45. Extra-axial chordoma of the anterior chest wall

Ben Davies, Royal National Orthopaedic Hospital; Jakub Jagiello, Royal National Orthopaedic Hospital; Harriet Branford White Royal National Orthopaedic Hospital Chordomas are rare malignant tumours of primitive notochordal origin. They typically present within the axial skeleton, causing pain and are locally aggressive.

A rare subset of chordomas (2.3%) have been reported to solely originate outside of the spine and these are known as extra-axial chordomas. Due to the rarity of the condition, there is a paucity of reported cases of extra-axial chordomas in the literature and rates of survival and recurrence are hard to predict. We report

a 28-year-old lady who presented with a 3-month history of a soft tissue swelling on her right anterior chest wall in the mid clavicular line overlying the 6th and 7th ribs.

MRI imaging revealed a well-defined soft tissue lobular abnormality with intermediate signal intensity on T1 and heterogeneously bright on fat-suppressed T2; with the differential including atypical lipomatous tumour, myxoma and liposarcoma. An ultrasound guided biopsy identified tumour cells diffusely expressing MNF116, brachyury and S100, which was consistent with a chordoma. A PET CT and MRI spine scan confirmed this as an isolated lesion with increased activity. Resection was advised following discussion within The London Sarcoma Service Bone and Soft Tissue sarcoma Multidisciplinary Team. Complete surgical excision of the tumour en bloc with segments of the 6th and 7th ribs was carried out. Histology confirmed a tumour with a myxoid stroma with chondroid areas and no evidence of de-differentiation. Due to limited data on prognosis and its rarity, we advocate radical resection of such tumours in the setting of a Sarcoma Unit.

46. A Proof-of-Concept Study using DCEMRI and Serum Proteomics to Investigate the in vivo Mechanism of Action (MoA) of trabectedin in Advanced Uterine Leiomyosarcoma (uLMS) and Non-Uterine Leiomyosarcoma or Liposarcoma within the Abdomen / Pelvis

Dr Robin Jones, Royal Marsden Hospital; Dr Swethajit Biswas2, Dr Yvette McGovern1, Galina Petrikova1, 1Royal Marsden Hospital, London, 2formerly Southampton University Hospital, Southampton Background: Trabectedin frequently achieves disease control in a wide range of sarcomas, despite having a low incidence of objective radiological response using RECIST criteria.

This suggests a novel, cytostatic, MoA rather than traditional cytotoxic activity.

Radiological observations clinically indicate that trabectedin might have cytostatic

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Aim and Objectives The primary objectives of this study are to look at changes in DCE-MRI enhancement and in plasma levels of circulating angiogenic markers and inflammatory cytokines with trabectedin treatment.

Specifically we will look at reduction in KTrans and reduction in sVEGF, sIL-8 and circulating monocytes. Secondary objectives will look at relapse-free survival and timeto-disease progression.

Methods: This is a phase II, proof-of-concept singlearm non-randomized interventional study looking at inoperable patients with advanced or metastatic: uterine leiomyosarcoma (uLMS); non-uterine abdominal or pelvic leiomyosarcoma who received treatment with trabectedin, until confirmed progressive disease or treatment intolerance.

47. A pilot study of patient reported outcomes when treated with Trabectedin for unresectable and/or metastatic soft tissue sarcoma

Dan Stark, Leeds Teaching Hospitals

NHS Trust Leeds Institute of Cancer and Pathology, St. James’s University Hospital; Dan Stark, Leeds Teaching Hospitals NHS Trust Leeds Institute of Cancer and Pathology, St. James’s University Hospital; Maria Marples, Samantha Noutch, Tom Kaye; Karen Ingham, Johanne Sugden, Rob Carter. Leeds Teaching Hospitals NHS Trust Leeds Institute of Cancer and Pathology, St. James’s University Hospital Background: Trabectedin improves progressionfree survival in un-resectable advanced soft tissue sarcoma, with a low level of reported grade 3 symptomatic toxicity for many patients. However objective responses are infrequent, with most patients who benefit using traditional oncological response criteria show a delay in progression with stable tumour. Patient reported outcome measures (PROMs) are central to understanding the impact of cancer therapies that may shrink tumours or control their growth, and sometimes improve the length of life, but with less disruption of lifestyle, and of toxicities. It is very important we use PROMs to evaluate the balance between risks and benefits to understand the overall impact of the therapy.

Aim and Objectives To examine the feasibility of PROMs to inform clinical management in the use of trabectedin in soft tissue sarcoma, and to generate future hypotheses for larger scale testing.

Methods: Adult patients treated in Leeds with Trabectedin as either second or third line therapy for relapsed soft tissue sarcoma, at 1.5 mg/m2 every 3 weeks according to NICE guidance and local toxicity protocols and tumour assessments will be recruited. The EORTC QLQ-C30 questionnaire will assess patients’ QoL within the last seven days at set time points prior to clinical out-patient assessment. These scores will be used to examine clinical and patient-reported symptom trajectories during trabectedin

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effects and specifically possible antiangiogenic activity. Trabectedin has also been shown to have selective cytoreductive effects on peripheral circulating monocytes and TAMs. Specifically, trabectedin has been shown to reduce the monocyte population in a caspase-8 dependent manner and attenuate macrophage differentiation in myxoid liposarcoma and round cell liposarcoma (MRLPS) patients. In MRLPS and a variety of epithelial tumours, TAMs are known to secrete overlapping proangiogenic and pro-inflammatory factors.

Dynamic contrast enhanced MRI (DCE-MRI) is used to provide functional information about the state of tumour angiogenesis and vascularity. The technique produces a series of images signal intensity changes that can be plotted as a function of time, on a pixelby-pixel basis, thus providing spatiallyspecific information.

treatment, and the relation of PRO scores to changes in tumour dimensions and time-to-tumour progression. We will also evaluate the acceptability and burden upon patients and professionals of undertaking this reporting.

<1mm) was 9.5% . RIA recurred in 48% patients at a median of 7 months following excision , and 71% of these patients have died compared with 19% of those without recurrence. Disease-specific mortality was median 22 months from diagnosis.

Results will be reported in 2018, depending upon rates of recruitment.

Conclusions With the recent trend towards BCS the incidence of RIA is likely to steadily increase although it remains a rare phenomenon. Our data indicates that recurrence is associated with increased mortality. The latency between radiotherapy and developing disease means patients and primary care doctors should be well informed and educated to identify RIA early and refer.

48. Radiation Induced Angiosarcoma of the Breast – A Growing Challenge in the Era of Breast Conserving Surgery

Philippa Jackson, Leeds Teaching Hospitals; Ian M Smith, Leeds Teaching Hospitals; Fiona Langlands - Leeds Teaching Hospitals Lauren Taylor - Sheffield Teaching Hospital William Merchant - Leeds Teaching Hospitals Raj Achuthan - Leeds Teaching Hospitals Introduction Radiation-induced angiosarcoma (RIA) of the breast is a rare disease. However, as breast conservation surgery (BCS) rates increase, a growing number of women are receiving breast radiotherapy.

Methods Patients diagnosed with RIA from 2005 to 2017 were identified through sarcoma and pathology databases. Basic patient demographics, details of the primary breast cancer and treatment, and diagnosis and management of RIA were recorded. Patients with non-RIA were excluded.

Results Thirty-one patients were identified in the 12-year period, complete data was available for 26. Median age at diagnosis was 70 years, with a median latency between radiotherapy and developing RIA of 86 months. Treatment of the primary breast cancer was wide local excision (WLE) and radiotherapy (40 Grays in 15 fractions) in 26 patients. Nine patients received a radiation boost. Surgical treatment of RIA constituted mastectomy +/- pectoralis excision in 26 cases. Incomplete excision rate (margins

49. Knowledge and attitudes towards Adolescent and Young Adult (AYA) Oncology amongst cancer care providers in Ireland

Scheryll Alken, St James’s Hospital; Dr Deirdre O’Mahony, Cork University Hospital; Dr Cormac Owens, Our Lady’s Children’s Hospital Crumlin; Dr Cliona Grant, St James’s Hospital; Dr Michael Capra, Our Lady’s Children’s Hospital Crumlin; Dr Jane Pears, Our Lady’s Children’s Hospital Crumlin; Dr Charles Gilham, St Luke’s Radiation Oncology Network Some studies indicate AYA survival with cancer may be inferior to that of paediatric patients with similar cancers, possibly related (in part) to differences in access to centralised treatment. Many countries have dedicated AYA oncology centres; Ireland has no such formalised service. We sought to identify the knowledge and attitudes of cancer care providers in Ireland towards AYA oncology.

Method We compiled a 20-question survey, using the NICE Children and Young People with Cancer document (2014) as a reference.

Participants were asked their career stage and practice profile, and their AYA oncology

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Results In total, 20 responses were received with more expected by the time of the conference. Most responders were trainees.

Over 60% of respondents saw more than 75 new patients each year. 57% saw AYA patients regularly, with most seeing 0 – 5 patients each year. 100% of respondents saw tumours considered to be common “AYA tumours” regularly in their practice.

47% of respondents had made specific arrangements for the care of AYA patients in their practice. Only 14% of responders reported dedicated AYA training, with all that training outside Ireland.

Conclusion This survey provides a snapshot insight into AYA attitudes and knowledge of cancer physicians in Ireland. Physicians recognise those factors necessary for a successful programme, some making arrangements at a local level. Future development on a national level is imperative.

50. Staging rationale in soft tissue sarcoma

Richard Hutchinson, Newcastle Hospitals NHS Foundation Trust; Craig Gerrand, Newcastle Hospitals NHS Foundation Trust; Breanna Winger (Newcastle Hospitals NHS Foundation Trust) Paul Rodham (Newcastle Hospitals NHS Foundation Trust) Kenneth Rankin (Newcastle Hospitals NHS Foundation Trust)

routine CT screening of the abdomen and pelvis in STS. A retrospective review of patients with STS, from a single specialist sarcoma unit, over a 2-year peroid was completed. Histological type, primary tumour location, imaging used in staging and significant positive findings found on staging scans were recorded. 158 patients were included with a mean age of 61 years.

We found a broad range of histological types with sarcoma NOS, myxofibrosarcoma and leiomyosarcoma being the most common. 78% of patients were staged with a CT of the chest, abdomen and pelvis (CT CAP). Abdomino-pelvic metastatic disease was found in 5 cases (3%), of which 2 patients had no concurrent pulmonary disease. Significant lymphadenopathy within the abdomen or pelvis was found in 11 cases (7%), of which 8 had no lung disease.

38 patients with sarcoma of the upper extremity or trunk were staged with CT CAP, of which 3 had disease that would have been missed if staged with CT chest alone.

There is no consensus in the literature on whether CT CAP should be used routinely as part of staging a STS. In a small minority of patients CT chest alone would fail to identify extra-pulmonary metastases, potentially leading to inappropriate management.

51. Quality of life and reintegration into normal life after extremity soft tissue sarcoma surgery

Srikanth T, Green L, Wilshaw N, Steele KH, Tamimy MS, Raurell A, Ashford RU, East Midlands Sarcoma Service, Nottingham; Thulasi Srikanth

Current guidance on soft tissue sarcoma (STS) management states newly diagnosed patients should undergo a CT chest as part of routine staging, with a CT of the abdomen and pelvis to be considered in cases of lower limb disease.

In recent decades, limb salvage has become the favoured treatment of extremity soft tissue sarcoma (STS). Despite providing better functional and equal oncological outcomes, patients are still left with varying levels of functional disability after their treatment. The effects of this on quality of life can be devastating.

This study aimed to assess the value of

The aim of this study is to evaluate how Page 73

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experience, including any specific training.

The survey was anonymous and circulated using pre-existing email databases of medical & radiation adult and paediatric oncology trainees and consultants.

functional impairment impacts reintegration into normal life, in patients surgically treated for extremity STSs by the East Midlands Sarcoma Service. This pilot observational study was conducted on 26 patients with surgically treated extremity STS. Three outcome measures were used: Toronto Extremity Salvage Score (TESS), measuring perception of difficulty performing everyday tasks; University of California, Los Angeles (UCLA) activity score, comparing intensity of pre- and post-operative activity and Reintegration into Normal Living Index (RNLI), measuring patients’ ability to accomplish activities they want to do, to their satisfaction.

From the results of the TESS and RNLI, daily working activities are the tasks that individuals find most difficult and the area most dissatisfied with. A mean TESS score of 2.68 indicates ‘moderate to extreme’ difficulty in performing usual duties at work and working for the duration of their usual hours. The UCLA score indicated 46% of the cohort reported no change in their activity levels post-surgery compared to presurgery. Of those who reported change, the mean reduction in activity was by 2 levels.

We conclude that whilst personal-level activities are limited by surgery, work-life participation restriction is perceived by patients to have the biggest effect.

Aim: The present case series focuses on the surgical technique and management abdominal wall desmoid tumours.

Materials and methods: In 10 patients (nine female and one male) over 5 years, the median age was 37.5 years (range24-46 years). All cases are managed in Sarcoma multidisciplinary team setting. The surgical procedure was jointly performed by team of plastic and(or) general surgeons. All abdominal wall desmoid tumours underwent surgical resection via abdominoplasty type incision and reconstruction with synthetic mesh repair.

Results: The median follow-up period was 34 months (range 4- 58 months). There were no postoperative complications and no evidence of recurrence.

Conclusion: The use of abdominoplasty type incision for tumour resection involving the musculoaponeurotic tissue and the defect reconstruction with synthetic mesh restores optimal abdominal wall strength and good functional outcome.

52. Abdominal wall reconstruction after Desmoid tumour resection- surgical technique and management – A single centre case series

Vinay Itte, Leeds Teaching Hospital NHS Trust; Ian M Smith, Leeds Teaching Hospital NHS Trust; 1) Mr Ashish Magdum (Registrar Plastic surgery) Leeds Teaching Hospital NHS Trust 2) Mr Raj Achuthan (Consultant General Surgeon) Leeds Teaching Hospital NHS Trust Introduction Desmoid tumours are rare (3% of all

sarcomas) locally aggressive, benign fibromatosis that carry high recurrence rate following surgical excision.

Abdominal wall desmoid tumours involve musculoaponeurotic tissue that is vital for core abdominal strength and following resection of tumour, restoration of abdominal wall strength is vital for a good functional outcome.

53. Resection of Chest Wall Chondrosarcoma and Reconstruction with 3-D Printed Titanium Implant

Ira Goldsmith, Morriston Hospital, Swansea, Wales. Peter Llewelyn Evans, Morriston Hospital, Swansea, Wales; Heather Goodrum, Morriston Hospital, Swansea, Wales; James Warbrick-Smith, Morriston Hospital, Swansea, Wales; Thomas Bragg, Morriston Hospital, Swansea, Wales

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Methods: A 76 year male presented with an enlarging right anterior pectoral mass of eight months duration. A biopsy confirmed chondrosarcoma arising from the right costal cartilage. This required an en bloc resection of the tumour with resection of the right pectoral muscles, the second to the fourth ribs, the intervening intercostal muscles and the right hemi-sternum. A multidisciplinary approach was adopted for the rigid and soft tissue reconstruction of the resulting defect. A custom-made titanium implant was designed, based on 3-D computed tomography scan reconstruction (will be described in detail at presentation), and the implant constructed using 3-D printing technology in the UK for the first time. At surgery, following resection, the large defect was reconstructed with placing a Gore-Tex patch and anchoring the implant to the fragments of the healthy ribs laterally and the hemisternum medially.

Finally, the surgical site was covered with a latissimus dorsi flap. Results: The postoperative course was uneventful.

Histology confirmed chondrosarcoma with clear microscopic margins. At follow-up there was no discomfort, pain, displacement or paradoxical movement and the patient was fully mobile.

Conclusion: Our case demonstrates that a 3-D printed custom-made titanium chest wall implant is feasible and safe with a satisfactory result making this a good surgical choice for reconstruction of large chest wall defects.

Dr Iain Macleod, Nottingham University Hospitals NHS Trust; Dr Cheika Kennedy, Nottingham University Hospitals NHS Trust; Dr Julia Fairbairn - Nottingham University Hospitals NHS Trust Dr Tom McCulloch Nottingham University Hospitals NHS Trust Peripheral nerve sheath tumours (PNST) are common soft tissue tumours, but imaging features are often non-specific. We present our experience of 120 benign and malignant cases managed through the regional sarcoma service over the last 7 years. All cases have been biopsy confirmed, and have been identified by a retrospective search of the histology database. The radiology has been reviewed independently by at least two radiologists within the MDT, and assessed for the presence or absence of â&#x20AC;&#x2DC;classicâ&#x20AC;&#x2122; PNST features, as well as any other salient imaging findings. From this, we can determine the frequency of findings on CT, MRI and ultrasound and discuss what features should suggest a diagnosis of PNST. We also examine whether it is possible to distinguish between benign and malignant tumours, or between the various different histological subtypes of benign tumours. This allows us to suggest an imaging strategy that aims to optimise the patient journey through the diagnostic process, and make efficient use of the limited resources available to a sarcoma service.

55. The cost of centralising sarcoma services for patients, a single centre experience.

Karen Oâ&#x20AC;&#x2122;Meara, Royal Marsden NHS Foundation Trust; Kelly McKibbin, Royal Marsden NHS Foundation Trust; Alison Dunlop, Angela Teague, Charlotte Benson, Aisha Miah.

NICE guidelines dictate that specialist sarcoma services are centralised. The access to centralised multidisciplinary teams and specialist services for rare cancers has

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54. PNSTs: our experience Background: Reconstruction of chest wall defects following resection of chest wall sarcoma is complex. We present a case of an adult male who underwent surgical resection of a chest wall chondrosarcoma followed by reconstruction of the resulting large defect with a custom-made titanium implant constructed using three-dimensional (3-D) printing technology in the UK for the first time.

been shown to improve patient outcomes.

However, it moves patients away from their local centres to access specialist care. In order to access specialist services, patients may have to travel long distances, multiple times while receiving interventions and treatment from the specialist teams. Our aim is to assess the financial impact of travelling to a central London hospital on our patient population. Following local committee approval, a prospective questionnaire was issued over one week to assess the impact of travel in relation to time and cost for patients. All patients who attended the sarcoma unit across the three modalities; radiotherapy, medical oncology and surgical were eligible for inclusion.

Over a one week period our centre reviews between 250-270 NHS patients in one hospital. Questionnaires were distributed on arrival for appointments and data was recorded anonymously. (The data collected from this audit is currently being reviewed and a follow up abstract will be available at the beginning of 2018) 56. Outcomes of patients receiving Irinotecan and Temozolamide for relapsed/refractory Ewing’s sarcoma (ES)

Helen Clarke, Queen Elizabeth Hospital Birmingham; Mariam Jafri, Queen Elizabeth Hospital Birmingham; Sumeet Mattharu, Queen Elizabeth Hospital Birmingham Sarah Coldicott, Queen Elizabeth Hospital Birmingham David Peake, Queen Elizabeth Hospital Birmingham Background Ewing’s sarcoma is a round cell sarcoma, commonly with histological evidence of EWS gene mutation in chromosome 22q12,1 with 80% of cases affecting those under 20. Second line treatment for ES is currently poorly evidence based, particularly in the adult population. Current 5 year survival for patients who have relapsed with ES is less than 30%.2 Methods We describe the outcomes of Irinotecan

and Temozolamide (IT) for the treatment of refractory and relapsed Ewing’s Sarcoma, in a group of 9 patients between the ages of 17 and 48, with a median age of 23. All patients were identified since 2005, 8 of whom had bone primaries and all of whom initially received VIDE chemotherapy. 2 patients presented with metastasis, 6 patients were on the REECUR trial3, 5 patients received IT second line and 4 patients third line. The median progression free survival (PFS) on IT was 6 months (2 – 13). The median overall survival (OS) from diagnosis was 119 months (18 to 145). It is important to note that of this group, two patients are currently ongoing with IT treatment. Within the limited data we have, there is no clear correlation for PFS on IT with age, previous chemotherapy, surgical intervention or metastasis at diagnosis.

Conclusion IT is associated with some encouraging results and our data supports the evaluation of this regime in the REECUR trial; there is a need to improve outcomes in this patient group.

57. Concurrent venous thromboembolism and lower limb soft tissue sarcoma – Problem and algorithm of management

Vinay Itte, Ian M Smith Leeds Teaching Hospitals NHS Trust; Mr Tony Virdee(Senior House Officer), Mr Ashish Magdum(SpR Plastic Surgery) Leeds General Infirmary, Leeds Patients with soft tissue sarcomas(STS) are at significant risk of developing venous thromboembolism(VTE). The condition remains underdiagnosed and inadequately treated. As a result, the morbidity and mortality due to deep venous thrombosis(DVT)and Pulmonary embolism(PE) is high and varies in the literature from 5.2to 14.8%. In addition, the management of VTE in STS is complex due to the effects of malignancy and its treatment. The retrospective study aims at investigating the incidence of VTE including DVT and PE in lower limb STS.

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Methods and materials â&#x20AC;&#x201C; 204 patients presented with lower limb STS over a 5-year period (2011-2016). Out of these, six cases presented with coexisting VTE underwent pre-operative inferior vena cava(IVC) filter, mechanical thromboprophylaxis and postoperative anticoagulation. The outcome measures include post-operative VTE and tumour recurrence. Â Based on our unit experience and review of the current evidence, we propose an algorithm and also suggest to prescribe post-operative extended thromboprophylaxis in all patients with lower limb soft tissue sarcomas.

59. MRI characteristics of Atypical Lipomatous Tumours

58. Rate and Patterns of Local Recurrence Following Radical Treatment of Soft Tissue Sarcoma

Rebecca Shakir, Oxford University Hospitals NHS Foundation Trust; Sally Trent, Oxford University Hospitals NHS Foundation Trust Local recurrence occurs in up to 20% of patients following radical treatment of soft tissue sarcoma with radiotherapy and surgery1. The cause of recurrence can be broadly categorised into inadequate clinical target volumes (CTVs), insufficient dose and intrinsic tumour radioresistance.

Recurrences due to inadequate CTVs tend to result in edge of field recurrences, and are more easily avoided by modification of radiotherapy technique than those which occur in field, where the dose delivered is limited by normal tissue tolerance. We present data from 187 patients with soft tissue sarcoma who underwent neoadjuvant or adjuvant radiotherapy in Oxford between 2008 and May 2016, 18 of

whom (9.6%) experienced a locoregional recurrence (LRR). 50% (9) of these were in field, with 28% (5) on the edge of field and 17% (3) outside of the treated volume (1 plan unavailable for review). Recurrence rate was similar regardless of location of disease. 15 of the patients who had local recurrence received adjuvant radiotherapy (LRR 11.7%, n=128), compared to 3 in the neoadjuvant group (LRR 5.01%, n=59). No subtype was dominant in the LRR group, with myxofibrosarcoma (5) and pleomorphic sarcoma (5) the most represented. This data supports our current contouring technique, but ongoing review of the cases where LRR was at the edge of field will help us to optimise margins to ensure adequate coverage of microscopic disease.

Reference: 1. Local recurrence and survival in soft-tissue sarcomas. Karakousis C.P. et al.

Annals of Surgical Oncology (1996) 3;3:255260

Onkarpreet Jassel, UoN; Robert Ashford, East Midlands Sarcoma Service; O Jassel (UoN), S Aggarwal (East Midlands Sarcoma Service), R Ibrahem Adam (East Midlands Sarcoma Service), TA McCulloch (East Midlands Sarcoma Service), MS Tamimy (East Midlands Sarcoma Service), KH Steele (East Midlands Sarcoma Service), A Raurell (East Midlands Sarcoma Service), RU Ashford (East Midlands Sarcoma Service) Background Atypical Lipomatous tumours (ALTs) are a type of lipomatous tumour that are classified as WHO intermediate soft tissue tumours that are locally aggressive but do not metastasize. Marginal excision is the standard treatment for these tumours.

The aim of our study was to characterise the pre-resection MRI appearances of histologically proven ALTs. Methods This is a retrospective review of histologically proven, surgically treated,

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The study looked at variable factors including patient comorbidities, tumour characteristics and operative procedures, and correlated that to the risk of developing VTE. We also propose an algorithm for the management of cases presenting concurrently with VTE in STS.

extremity and trunk wall ALT patients.

Patients were identified from a pathology database and then electronic case notes and digital images were reviewed.

Results 49 patients who were surgically treated by the East Midlands Sarcoma Service from 1997 to 2017 were identified. 29 patients were male and 20 were female (59.2% and 40.8% respectively), the ages ranged from 27 to 90 years of age, with a mean of 64 years. They were more frequent in the lower limb than in the upper limb, trunk wall or in the head or neck (61.2% vs. 16.3%, 18.4% and 4.1% respectively).

Most of the ALTs were located in the deep compartment (92.50% vs. 7.50% in the superficial compartment). The mean maximum dimension of tumour showed to be 115.5mm, with lesions ranging from 40mm â&#x20AC;&#x201C; 385mm. 40.7% of the cases noted intratumoral vessels and 44.4% noted the presence of septa. 2 patients developed local recurrence.

bone.

Methods Data was collected on patients with confirmed histological diagnoses of primary long-bone osteosarcoma or Ewing sarcoma who underwent resection or limbsalvage surgery from 2007-2015. Data was obtained from Infoflex, correspondence, histopathology and radiology reports.

Results 169 patients were included (ages - range 1-76, Mean - 18). The intraosseous surgical margin was complete in 163 patients (96%). It was intra-lesional in 4 cases (2%) and 2 cases had insufficient data. Mean intraosseous margin - 39.7mm, range 1mm to 130mm. In 5 patients the margin was =< 5mm. There were 19 cases (11.2%) of local recurrence within 2 years of diagnosis. Of these, 13 (7.7%) recurred in soft tissue and 6 (3.6%) in bone. Of the local recurrence in bone, all the intraosseous margins were complete. In 5/6 of these cases, soft tissue margins were marginal (<1mm of fibrous tissue) and in 4/5 cases there was a poor response to neo-adjuvant chemotherapy.

Conclusions ALTs were 12 times more common in the deep compartment compared to the superficial. Septa and vessels were present in nearly half the cases. The presence of these 3 features make ALT likely on MRI. 60. Is whole bone MRI safe for planning resection or limb salvage surgery in long bone osteosarcoma and Ewing Sarcoma?

Mr James Barnett, Royal National Orthopaedic Hospital, Stanmore; Dr Asif Saifuddin, Royal National Orthopaedic Hospital, Stanmore; Panagiotis Gikas Royal National Orthopaedic Hospital, Stanmore

Conclusion Whole bone MRI is a safe method to plan surgical resection with 96% of patients having clear intraosseous margins. The incidence of local recurrence in bone was 3.6% within 2 years of diagnosis. However, this is multifactorial and where soft tissue margins are complete, there was only a 0.6% chance of local recurrence in bone.

61. Wild type GastroIntestinal Stromal Tumours: an adult pooled analysis compared with paediatric controls

Introduction Appendicular osteosarcoma and Ewing sarcoma are treated surgically by limbsalvage or amputation, with whole bone MRI used pre-operatively to plan resection level.

This study aimed to determine the safety of this practise by assessing intraosseous surgical margins and local recurrence in

Cillian Clancy, Dept Surgery, Tallaght Hospital; Ridgway PF, ISG/ Tallght Hospital/ TCD Dublin; Bhatt N.R1, Clancy. C1, Crotty P2, Gillis. A1,3, Ridgway P.F1,3 1: Department of Surgery, Tallaght Hospital/ TCD, Dublin 24 2: Department of Pathology, Tallaght Hospital/TCD, Dublin 24 3: Irish Sarcoma Group

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Methods MEDLINE and SCOPUS databases, combined with a local series, were searched using standardised terms over a 16 year period.

Studies required follow-up data on adult patients with WT GISTs to be included.

Patients with known NF-1 were excluded.

Data on paediatric patients with WT GISTs was recorded for comparison. Kaplan-Meier analysis was used to depict survivals (Log Rank comparisons).

Results Eighty-four adult patients from 15 studies were compared with thirty-eight pediatric patients from five studies. Apart from age, characteristics of adult GISTs were similar to the pediatric group. There was no statistically significant difference in the survivals between both groups (p= 0.2) although there were a small percentage of adult WT GISTs recurring within 3 years versus 0% in the paediatric sample.

Median overall survivals were not reached.

Reported Mean OS was 32 years (26-38 years); median DFS was 10 years and 5-year OS was over 85%.

Conclusion Overall survival in adults with WT GISTs is favourable compared to other adult GIST subtypes, and similar to a paediatric GIST population. Larger sample sizes are needed to elucidate the role of adjuvant strategies in higher risk adult WT GIST. 62. Demographics and outcomes of extracranial chordomas and

chondrosarcomas treated with high dose external beam photon radiotherapy: The UCLH centre experience

Dr Pei LIM, UCLH; Dr Franel LE GRANGE, UCLH; Dr Beatrice SEDDON Dr Mahbubl AHMED Chordomas and chondrosarcomas are rare bone malignancies which are locally invasive with high recurrence rates despite surgery.

Patients are usually offered adjuvant radiotherapy or definitive radiotherapy if the tumour is unresectable. These tumours are considered relatively radio-resistant, requiring high doses of radiation to achieve local control, which is a technical challenge to deliver due to the risk of damaging critical surrounding tissues such as spinal cord, nerve roots and bowel.

We report the characteristics and outcomes of patients who received high dose external beam photon radiotherapy (>45Gy EqD2) for extracranial chordoma and chondrosarcomas treated at UCLH. Between December 2002 and September 2017, 43 patients with pathologically proven extracranial chordomas (n=23) and chondrosarcomas (n=20) were treated. The median age of patients at radiotherapy was 64 years (range 30-85 years). The mean follow up time was 33.4 months (range 1.5114.5 months). The radiotherapy indications were primary treatment (n=6 chordoma, n=4 chondrosarcoma), adjuvant (n=11 chordoma, n=12 chondrosarcoma), high dose palliative (n=3 each), neoadjuvant (n=1 each). In the chordoma cohort, the acturial 2 and 5-year local recurrence-free survival, 5-year distant progression-free survival and 5-year overall survival rates were 72%, 38%, 50% and 55%. In the chondrosarcoma cohort, the rates were 35%, 24%, 28% and 22% respectively. We observed two Grade 3 bowel toxicities in the chordoma cohort.

These results indicate that high-dose photon radiotherapy is of benefit as a treatment option for patients who are not suitable for overseas proton therapy referral

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Background Wild type (WT) form a minority (approximately 7%) of adult Gastrointestinal Stromal Tumours (GISTs). WT GISTs represent a management conundrum both in perceived prognosis as well as the role of adjuvant therapies. We sought to characterise clinicopathological outcomes of adult WT GIST based on a pooled analysis of published data compared with WT GISTs in the pediatric population.

63. Working together to ensure sarcoma patients receive the high-quality information they deserve.

Hannah Birkett, Bone Cancer Research Trust Background: At the Bone Cancer Research Trust (BCRT) we are constantly speaking to patients, family members and healthcare professionals (HCP’s) to gain a better understanding of what information and support patients with bone sarcoma want and need. It is crucial we work together in this way to produce high-quality resources and fill gaps in information.

Geraint Davies, Robert Jones & Agnes Hunt Orthopaedic Hospital NHS Foundation Trust; Paul Cool, Robert Jones & Agnes Hunt Orthopaedic Hospital NHS Foundation Trust Predicting ambulation outcomes following lower limb amputation using the Amputation Mobility Predictor assessment tool.

Methods: Harry has an Operation: A Guide for Children with Bone Sarcoma is an illustrated children’s storybook published by BCRT in October 2017. It is written for parents, carers and HCP’s to read with children in order to help them understand their limb-sparing surgery or amputation. This resource was produced in collaboration with 10 HCP’s from a range of specialisms; including oncologists, surgeons and physiotherapists, to allow the improvements and detailed knowledge required for this resource to be incorporated. Furthermore, our most recent publication, ‘A Step-By-Step Guide for Patients’ was developed with the guidance of our nurse-led focus-group.

Measuring the Impact/Discussion: The first ever children’s resource for bone sarcoma and our interactive patient guide are a credit to this collaboration of charities, HCP’s, patients and families. This presentation will detail the development and review of BCRT’s most recent publications while expanding on the use and feedback of these resources across the UK, with the aim to inform HCP’s and patient representatives on the ways in which they can help ensure patients of all ages receive the information they deserve.

64. Ambulation outcomes following lower limb amputation

Background Following the increase in the number of patients requiring lower limb amputation, a dedicated clinic was commenced at The Robert Jones & Agnes Hunt Orthopaedic Hospital, Oswestry in 2013. The clinic was enhanced to include non tumour patients who have had a referral for a potential amputation for chronic infection, trauma and deformity. The Amputee Mobility Predictor (AMP) Score is an instrument that is used as part of the overall assessment to assess determinants of the lower-limb amputee’s ability to ambulate following surgery.

Objectives Compare the Amputee Mobility Predictor scores completed pre-operatively and postoperatively with a prosthesis in situ.

Method Data was collected prospectively from 2014 to 2017. A total of 36 patients attended clinic and were assessed using the Amputee Mobility Predictor. 13 patients did not have surgery following assessment, 11 patients completed the assessment postoperatively only, 6 patients completed the assessment pre-operatively only and 6 patients completed both pre and post op assessments.

Results The pre and post-operative Amputee Mobility Predictor scores were analysed.

Out the 6 patients that completed both assessments, 5 patients had higher scores following surgery with a prosthesis in situ (mean increase 11%). There was only a marginal difference in the patient’s score that demonstrated a lower score post-

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Conclusion The Amputee Mobility Predictor has demonstrated that with our small cohort, it has successfully predicted post-operative amputation ambulation outcomes. Further analysis is required with a larger group sample.

65. Regorafenib in the third line setting for gastrointestinal stromal tumours (GIST): A single centre experience

Lucy McAvan, University Hospitals Birmingham NHS Foundation Trust; Mariam Jafri, University Hospitals NHS Foundation Trust; Shaikh Muhammad Faisal Rana, David Peake University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.

INTRODUCTION The discovery of mutational targets in GIST has changed the face of management of this malignancy and significantly altered outcomes for patients. Regorafenib, a multi-targeted tyrosine kinase inhibitor, has approval for treatment of GIST following progression on or intolerance of Imatinib and Sunitinib. In a phase 3 randomised trial Regorafenib improved PFS from 0.9 to 4.8 months with an acceptable toxicity profile1. Here we report the outcomes of patients treated with Regorafenib for GIST at University Hospitals Birmingham, a large teaching institution.

METHODS Patients with GIST who had received Regorafenib were identified. Data was collected on demographics, disease characteristics, survival and details of Regorafenib treatment.

RESULTS 6 patients were identified who had commenced Regorafenib between October 2014 and June 2017. There was a mixture of primary sites of disease and mutations. All patients had previously received Imatinib,

median time on treatment 24 months, and Sunitinib, median time on treatment 6 months. Responses included 1 partial response (by Choi criteria) and 3 stable disease, objective response rate 66%. At the time of analysis 3 patients had died and 2 had ongoing treatment with Regorafenib.

1 year progression free survival rate was 50%.

Regorafenib was well tolerated with no reported grade 3 toxicity. 1 patient stopped treatment due to intolerance and poor quality of life, 2 patients required dose reductions.

CONCLUSION Our patient population did better than the reported outcomes in the trial with minimal toxicity indicating a promising future for Regorafenib in the treatment of GIST. 1. Demetri GD, Reichardt P, Kang YK, et al. Efficacy and safety of Regorafenib for advanced gastrointestinal stromal tumours after failure of Imatinib and Sunitinib (GRID): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet 2013;381:295-302.

66. Should we be offering pre-op radiotherapy to our sarcoma patients?

Timothy Mitchell, Beatson West of Scotland Cancer Center; Fiona Cowie, Beatson West of Scotland Cancer Center; Ioanna Nixon Beatson West of Scotland Cancer Center Aim: To evaluate measures of response to preoperative radiotherapy (RT) for limb Soft Tissue Sarcoma (STS).

Methods: Retrospective review of 20 sequential patients receiving limb RT for nonmetastatic histologically proven STS from the local radiotherapy database. Response to preoperative RT was assessed using volume change on MRI and pathological percentage necrosis. Pathology retrospectively reviewed by 3 sarcoma pathologists for necrosis rate. All patients

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operatively (2%)

treated with standard dose and fractionation using either conventional or VMAT technique.

Results: 11 myxoid liopsarcoma, 4 pleomorphic, 2 synovial, 2 spindle and 1 MPNST. Size range (longest dimension), 40 to 180mm. The range of change between pre and post-op MRI volumes was -665 mls to + 3213 mls.

The pathological responses after RT ranged from 55 to 99%. Good inter-observer agreement for non myxoid liposarcoma, less agreement for myxoids.

Conclusion: Pre operative RT did not consistently decrease tumour volume or induce significant pathological response.

Differences between results from three pathologists reporting necrosis/ response warrant further investigation.

Post operative complications after pre operative radiotherapy have been a local concern, however review of post operative complications has resulted in debate over scoring/ grading, rates of complications cannot be included until this is agreed locally. The overall benefit of pre operative RT has been questioned following a number of post operative complications. Pathology and radiology correlated fairly well as a marker of response, but less convincingly than expected. How can the risks and benefits for pre versus post operative radiotherapy be best evaluated to inform decision making?

67. Multifocal Dermatofibrosarcoma Protuberans --- A Rare Case Report

Qi Yin, Royal Liverpool University Hospital; Professor Tim Helliwell Department of Pathology, Royal Liverpool University Hospital Dermatofibrosarcoma protuberans (DFSP) is an uncommon superficial soft tissue malignancy that typically presents with local invasion but rarely metastasizes. It constitutes 1% of all soft tissue sarcomas.

Majority of patients with DFSP are single lesion. Herein we report a rare case of multifocal DFSP. A 64-year-old lady presented with 12-month history of a skin lesion of left knee. She underwent surgery by general surgeon under LA and the histology confirmed DFSP with positive margins. She was subsequently referred to Liverpool Sarcoma Service for further management. The staging CT showed no evidence of metastasis, but local MRI scan showed an enhancing nodule. She underwent further local wide excision under GA and histology confirmed small residual DFSP (13x 13 x 8mm) with clear margins. Three years later, she presented with another skin lesion of right lower leg.

She reported that she has had this lesion for 2 months and the lesion has increased in size. The ultra-sound scan demonstrated a vascular lesion (11mm). Subsequently she underwent local wide excision. The histology confirmed DFSP with clear margins. She had further staging CT scan of chest, abdomen and pelvis, which showed no evidence of metastasis. Therefore the two distinct lesions of bilateral lower legs have to be regarded as multifocal DFSP. Conclusion: Although DFSP has a marked tendency to recur locally after surgical excision, rare cases metastasize to regional lymph nodes or distant sites. This is a very rare multifocal DFSP case report and will raise awareness of possible multifocal disease for DFSP. 68. Surgical excision margins in soft tissue tumours â&#x20AC;&#x201C; An audit of 91 patients

Christian Gray Stephens, Royal National Orthpaedic Hospital; William Aston, Royal National Orthpaedic Hospital; Benjamin Davies Andrew Johnston Background: MDT discussion of soft tissue tumour referrals is intended to reduce the risk of misdiagnosis and suboptimal excision resulting in unsatisfactory outcomes.

This study aimed to identify any factors associated with the MDT and surgical pathways that may result in suboptimal

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Methods: A retrospective review was performed of all soft tissue tumour excisions at a tertiary orthopaedic oncology centre (between 16/7/17 and 15/9/17). Factors examined included radiological opinion, preoperative biopsy, MDT outcome, operating surgeon grade and excision margin.

Results 91 excisions were reviewed; the mean age was 49.8, M:F 48:43 and 27(27%) were found to be malignant. 25 radiologically diagnosed cases of PVNS, GCT and lipomatous lesions were excised. Of the 66 others, 23 were biopsied preoperatively and a further 12 were recurrences of known disease. Leaving 31 cases treated with excsion biopsy. In 12 cases the treatment pathway was felt to be sub optimal. Root cause analysis identified 14 issues; 11 related to the treatment and 3 to the MDT pathway. Sub analysis showed that Registrar/Fellow grade surgeons accounted for a higher proportion of excision biopsies and issues related to treatment.

Conclusion This highlights the importance of clearly documented MDT decisions, and decision making related to the potential histological diagnosis of the tumour and the experience of the operating surgeon.

69. Characteristics of patients diagnosed with angiosarcoma presenting to a Sarcoma Centre

Gurvin Chander, University Hospital Birmingham; Mariam Jafri, University Hospital Birmingham; Samuel Ford, Hassan Douis, Haider Abbas David Peake, All at University Hospital Birmingham Background Angiosarcomas are rare but very aggressive sarcomas. They are associated with radiation exposure e.g. for breast cancer or in sun-exposed sites. The evidence base for

managing these patients is limited and typically in metastatic/locally advanced disease paclitaxel is utilized.

Methods Patients with angiosarcoma presenting between Jan 2013 and Nov 2017 were identified using computerized clinical records. Data were collated on; the site of angiosarcoma, previous diagnoses, surgical management, chemotherapy and tolerance to chemotherapy.

Results 48 patients were identified. The median age at diagnosis was 71 (56â&#x20AC;&#x201C; 85 years). The most common sites of presentation were; breast (34), face (4), scalp (2), chest wall (2), chest wall (2) other (4). 37 patients (77%) had previous exposure to radiation. The median time for angiosarcoma to develop after a breast cancer was 8 years. 83% of patients had a surgical resection. Only 18 (38%) patients had chemotherapy for metastatic/locally advanced angiosarcoma (paclitaxel (12), doxorubicin (4), gemcitabine/docetaxel (2)). The median number of cycles was 2.97, 5 progressed requiring second line chemotherapy.

Patients received doxorubicin (3), trabectedin(1) and gemcitabine/docetaxel (1) in the second line. The median overall survival of patients was 39 months.

For those receiving paclitaxel median progression free survival (PFS) was 8.5 months (range 1-13).

Conclusions Surgical treatment remains the mainstay of managing angiosarcoma. Locally advanced and metastatic angiosarcoma is associated with a poor prognosis. Chemotherapy was associated with poor PFS, this maybe due to the relatively older age of this patient cohort. Better treatments are required for advanced disease.

70. The use of mifamurtide in completely resected high grade osteosarcoma in two University Hospitals

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outcome.

Haider Abbas, University Hospital Birmingham NHS Foundation Trust; Mariam Jafri, University Hospital Birmingham NHS Foundation Trust; Ivo Hennig, Nottingham University Hospitals NHS Trust; S Coldicott, University Hospital Birmingham NHS Foundation Trust; D Peake, University Hospital Birmingham NHS Foundation Trust Background Osteosarcoma is the most common primary tumour of the bone. Treatment with multi agentt perioperative chemotherapy and surgery is associated with long term survival in 60%-70% with localized disease.

However, the prognosis remains poor for unresectable primary disease or metastatic disease. Mifamurtide given along with standard adjuvant chemotherapy in highgrade osteosarcoma patients aged between 2 and 30 years has improved 6-year overall survival from 70% to 78%. We describe our experience of this regime in two sarcoma units.

Methods Patients diagnosed with high grade osteosarcoma at Queen Elizabeth Hospital Birmingham and Nottingham University Hospital between April 2011 and February 2016 were identified through computer records. Their records were retrospectively evaluated for: demographic data, histology, treatment.

Results 26 patients were identified, 21 patients were aged between 2-30 years and 5 were older than 30., 24 patients received MAP chemotherapy, methotrexate was omitted from cycle 2 in one patient due to renal toxicity. All patients underwent surgical excision, 25 patients had clear postoperative margins and 9 patients had >90% chemotherapy associated necrosis. All 26 patients received Mifamurtide. 10 received all 48 doses, 3 had between 40 to 47 doses and the rest had 5 to 33 doses. The median overall survival of the cohort was 85 months.

Conclusion Mifamurtide is the only drug to have shown

improvement in overall survival in patients with high grade osteosarcoma and has manageable toxicities. Our data has shown that >90% of patients received >40 doses of Mifamurtide. Our survival data was comparable to published series.

71. Imaging appearances of gastrointestinal stromal tumours (GISTs)Is there a correlation with precision computer tomography (CT) findings, genomics and outcomes?

Dr Balraj Dhesi, University Hospitals Birmingham NHS Foundation Trust; Dr Hassan Douis, University Hospitals Birmingham NHS Foundation Trust; Dhesi B (1), Abbas H (2), Jafri M (2), Desai A (3), Ford S (3), Tanniere P (4), Douis H (1) Department of Clinical Radiology (1), Clinical Oncology (2), Surgery, and Histopathology (4).

Background: GISTs are associated with specific genomic alterations. CT is routinely used to assess GISTs, this study explores whether specific CT findings can predict genomic alterations and survival.

Methods: We retrospectively analysed CT scans from 48 patients diagnosed with GIST between May 2009 and November 2015. Imaging features including enhancement, volume and necrosis were correlated with survival and genomic alteration.

Results: 9 patients had wild-type GIST; 5 gastric and 4 small bowel. The mean tumour volume(MTV) was 366cm3. 2 had calcification. 2/9 had late metastases.

5 patients with exon 9 mutations were identified; 2 gastric and 3 small bowel.

MTV was 227cm3. 2 showed areas of calcification. 3/5 developed late metastases. 15 patients had exon 11 mutations; 9 gastric, 4 small bowel and 2 colonic tumours. MTV was 442cm3. 3 tumours showed calcification. 1/11 tumour

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Conclusion: CT findings in GISTs may be used as important imaging biomarkers. However, this finding requires further evaluation in a larger patient cohort.

72. Review of Deep Infections Associated with Endoprosthetic Replacement

Ian Crowther, Newcastle upon Tyne Hospitals NHS Foundation Trust; Martin Marsh, Newcastle upon Tyne Hospitals NHS Foundation Trust; Kenneth Rankin, Craig Gerrand; Newcastle upon Tyne Hospitals NHS Foundation Trust Massive endoprosthetic replacement is an important treatment for musculoskeletal tumours. While the majority are successful, infection remains a devastating complication. We aimed to retrospectively review our experience of endoprosthetic infection. Between 2003 and 2017, 95 patients underwent endoprosthetic replacement of a major bone. 11 (12%) developed deep infection, which was confirmed at an average of 367 days (range 23-1460) after surgery. The diagnosis was Osteosarcoma (5), Ewings Sarcoma (1), Chondrosarcoma (1), Adamantinoma (1), Giant cell Tumour (1), Malignant Fibrous Histiocytoma (1) and Melanoma (1). The most prevalent anatomical site was the Distal Femur (4), followed by Proximal Femur (3), Proximal Tibia (2), Diaphysis Tibia (1) and Proximal Humerus (1).

At the time of diagnosis, the majority of patients had clinical signs of infection including pain, swelling and erythema, with 5/11 having an established sinus.

Staphylococcus epidermidis (5) and Staphylococcus aureus (3) were responsible for the majority of the infections with one polymicrobial infection. Debridement was the most common initial surgery but was only successful in one case. Three patients required amputation following attempts at limb salvage and two patients remain on long term antibiotic suppression.

Patients underwent an average of 4 operations (range 1-8) to achieve control of the infection. The average time from diagnosis of infection to clinical confidence in the successful eradication of infection if achieved was 956 days.

Deep endoprosthetic infection remains a significant challenge and often requires several procedures. We would recommend early and open discussion with patients about treatment and outcomes and a MDT approach.

73. 5 year experience of sarcoma at UHL

S Jennings, Department of Surgery, University Hospital Limerick, Limerick, Ireland; M Flanagan, Department of Surgery, University Hospital Limerick, Limerick, Ireland Introduction: Soft tissue sarcomas are a rare heterogenous group of malignant tumours that represent approximately 1% of adult malignancies. The aim of this study was to describe the 5-year experience including demographic and histological subtypes of primary sarcomas treated in a general and orthopaedic sarcoma centre at University Hospital Limerick (UHL).

Methods: Data including patient demographics, presentation and tumour location, was acquired from a prospectively maintained

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showed late metastasis. 15 patients with exon 18 PDGFRA mutations were identified, all gastric in origin. MTV was 594cm3. No tumours showed calcification.

Only 1/15 tumours showed metastasis, later in the clinical course. CT features of necrosis was associated with poorer survival(p<0.0005). 18 had homogeneous, 14 had heterogeneous, and 17 had rim enhancement. Heterogeneous enhancement was associated with worse prognosis (ns).

Due to small numbers, statistical differences in survival due to genomic differences and calcification were not identified.

database of all soft tissue tumours referred to a surgical oncology department from 2012-2017. Further data including histological subtypes, treatment and follow up and data relating to extremity sarcoma was acquired from Hospital In-Patient Enquiry (HIPE) data, histology reports and chart review.

Results: During the study period January 2012 December 2017 20 patients presented with sarcomas. A total of 13 soft tissue sarcomas presented to a surgical oncology department, while 7 presented to an orthopaedic department. Males comprised 65% of the cohort. The most common sites were lower limb, upper limb and chest wall.

Sarcoma subtypes included liposarcoma (3), myxofibrosarcoma (3), leiomyosarcoma (6), angiosarcoma (2), rhabdomyoscarcoma (2), synovial sarcoma, dermatofibrosarcoma protuberans, mesothelioma and unspecified sarcoma. The majority of patients received tumour directed surgery.

Conclusion: Sarcoma is rare and requires multidisciplinary input for optimum clinical outcomes. We identified leiomyosarcoma to be the most common sarcoma subtype to present to our unit. This is in keeping with trends for soft tissue sarcomas from the National Cancer Registry of Ireland (NCRI)1.

(1) - Oâ&#x20AC;&#x2122;Brien, K. 2014. SDPWK. Cancer trends No 25. Soft tissue sarcomas. National Cancer Registry. Available at: http://www.ncri.ie/ sites/ncri/files/pubs/Soft-Tissue-Sarcomas. pdf

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Implimentation of Nurse led Clinics

Caroline Pemberton and Jane Evans, Robert Jones and Agnes Hunt Orthopaedic Hospital The Musculoskeletal Tumour Unit treats patients with Bone and Soft Tissue Sarcomas, Bone Metastases, benign Bone and Soft Tissue Tumours and Tumour-Like conditions. The number of referrals to the unit have increased by 40% between 2012 and 2016. The challenges of meeting cancer targets and the increase in awareness by GP’s plus pressures on consultant time required a strategic review of service provision. Implementation of nurse led clinics was to provide a ‘one stop shop’ for urgent soft tissue referrals from GP’s under the two week rule. This includes an USS with a report and clinical assessment by the CNS’s Since it’s inception in 2016 the cohort of patient that CNS’s assess has extended to include, surgical discharges and long term surveillance of patients with soft tissue sarcomas. An audit was undertaken to assess patient satisfaction with the level of care, the speed and efficiency of the service. Nurse led activity has resulted in 35 consultant clinics being made available to accommodate more complex cases.

Nurse Specialist Support in Sarcoma services: an analysis in unscheduled workflow

efficiency of service delivered. Relatively little is known about workflow of such direct patient contact roles as they pertain to workforce planning.

Methods We sampled our prospective Sarcoma database at one centre over a 2.5-year period. Study period commenced on first interaction between CNS and patients in the lead up to the diagnosis. Time spent ensuring treatment bookings was excluded.

The study ended 30 days after completion of their definitive initial treatment, in order to reduce long follow up biases. Phone calls and ward visits were rounded to the nearest minute. Location/ Type of interaction was recorded. Quality of interactions were studied with a non-blinded questionnaire.

Data was analysed non- parametrically.

Results A sample was obtained from a database of 127 sarcoma patients presenting to Tallaght Hospital since 2015. All patients had positive views of the CNS-patient interactions. A median of 85 minutes were spent with each patient journey studied. Slightly more pre versus post treatment interactions were seen, but this was not statistically significant (Median 3 v 2; p=0.17). Surprisingly, a significantly less proportion of time (28%) was spent on inpatient interactions in contrast to pre-treatment (46%).

Una Hayden, ISG/ Tallaght Hospital; Paul F Ridgway, ISG/ TCD/Tallaght Hospital; Hayden U 1,2, Gillham C 1, O’Toole G 1, Gillis A 1,2, Ridgway PF 1,2 Institutions: 1: Irish Sarcoma Group 2: Tallaght Hospital/ TCD, Dublin 24, Ireland

Conclusions These data suggest that a significant amount of time is spent by the CNS in valuable direct patient contact. This key nursing role should be protected by clerical support to mitigate time spent in coordination roles.

Background Clinical Nurse Specialists (CNS) play a crucial role in supporting patients with Sarcoma diagnoses. Frequently, the nurse-patient interaction is a key determinant in the

Measuring the impact of Outpatient Physiotherapy intervention with a patient reported outcome tool, MYMOP

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Abstracts -National Sarcoma Forum

Abstracts - National Sarcoma Forum

Debbie Artis, Lynn Hopcroft, Leeds Teaching Hospitals NHS Trust Introduction MYMOP (Make Yourself Medical Outcome Profile version 2) is a validated patient driven quantitative tool to measure outcomes following physiotherapy intervention.

Conclusion MYMOP is able to detect an improvement in patient reported symptoms following a course of physiotherapy after muscle resection. Physiotherapy can improve symptoms which have been present for months or years.

Sarcoma UK support line update

The purpose of this study is to determine whether MYMOP is a useful tool to detect an improvement in patient symptoms following physiotherapy after sarcoma treatment.

Helen Stradling, CNS/Support Line Lead, Sarcoma UK

Method During the initial consultation the patient is asked to complete a baseline MYMOP questionnaire. The patient identifies up to two musculoskeletal symptoms and they rate the severity of these symptoms on a scale. The patient also identifies an activity of daily living that they would like to improve. After a course of physiotherapy, the MYMOP is repeated. The MYMOP tool states that a reduction in the overall score by one is indicative of worthwhile improvement following physiotherapy. This process was undertaken on a cohort of patients who were treated by the Sarcoma team.

Results 22 patients completed the outcome tool between Dec 2015 and September 2016.

All patients had previously had muscle resected at surgery. 12 patients had also received radiotherapy.

The median baseline MYMOP score was 4.7 (range 3.25-6.00). Following physiotherapy intervention, the median MYMOP score was 1.25 (range 0.0-3.75). This post intervention MYMOP score was significantly lower than the pre intervention MYMOP score (p<0.001, Wilcoxon Signed rank test).

The Sarcoma UK support line has now been operational for 2 years. In that time we have received 2372 contacts from 778 individuals.

The complexity of the contacts is increasing and the types of calls/emails are extremely varied. The service would like to share with the BSG examples of the types of contacts we have received and give an insight into things that we are being told. As Sam and I are both from clinical backgrounds we are aware of how much information people do/ do not retain after being given a cancer diagnosis and we find it interesting to hear what people take away from their clinical reviews.

The average time spent on telephone conversations with callers is 28 minutes which shows that we have the time to listen and discuss things at length which busy clinical teams do not. It has become very clear to us, as a team, that what people hear is not always what the clinical teams would have said, and so being able to go through with them what was said and try to unpick things for them can be challenging, but rewarding, for all.

On an individual patient basis, all patients demonstrated an improvement in their MYMOP score of at least 1.75.

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Annual Conference 2019 London SAVE THE DATE

26th & 27th February 2019 Royal College of Physicians, London Find out more at: www.britishsarcomagroup.org.uk

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Recommend as second-li ed treatment o ne ption â&#x20AC;&#x201C; BSG Guidel ines 20161

PharmaMar are proud to be the main sponsor again of the BSG 2018.

Yondelis prescribing information can be found on this exhibition stand.

1. Dangoor et al. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res (2016) 6:20 Date of preparation: January 2018 YON0118-1226