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SECOND EDITION

NIELSEN | ROSENBERG

DESHPANDE • HORNICEK • KATTAPURAM • ROSENTHAL

SECOND EDITION

G. Petur Nielsen, MD

Pathologist, Department of Pathology

Director of Electron Microscopy

Director of Bone & Soft Tissue Pathology

Massachusetts General Hospital Professor of Pathology

Harvard Medical School Boston, Massachusetts

Andrew E. Rosenberg, MD

Professor and Vice Chair

Director of Bone & Soft Tissue Pathology

Department of Pathology

Miller School of Medicine

University of Miami Miami, Florida

Vikram Deshpande, MD

Associate Pathologist

Department of Pathology

Massachusetts General Hospital

Associate Professor of Pathology

Harvard Medical School

Boston, Massachusetts

Francis J. Hornicek, MD, PhD

Chief, Orthopaedic Oncology Service

Co-Director, Center for Sarcoma and Connective Tissue Oncology

Massachusetts General Hospital

Director, Stephan L. Harris Chordoma Center

The Henry J. Mankin, MD, Endowed Scholar Professor

Harvard Medical School

Co-Leader, Dana Farber/Harvard Cancer Center Sarcoma Program

Boston, Massachusetts

Susan V. Kattapuram, MD

Associate Radiologist

Massachusetts General Hospital

Associate Professor of Radiology

Harvard Medical School

Boston, Massachusetts

Daniel I. Rosenthal, MD

Associate Radiologist-in-Chief

Massachusetts General Hospital Professor of Radiology

Harvard Medical School

Boston, Massachusetts

1600 John F. Kennedy Blvd.

Ste 1800 Philadelphia, PA 19103-2899

DIAGNOSTIC PATHOLOGY: BONE, SECOND EDITION

Copyright © 2017 by Elsevier. All rights reserved.

ISBN: 978-0-323-47777-2

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/ permissions.

This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein).

Notices

Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary.

Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility.

With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions.

To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein.

Publisher Cataloging-in-Publication Data

Names: Nielsen, G. Petur (Gunnlaugur Petur) | Rosenberg, Andrew, 1953-

Title: Diagnostic pathology. Bone / [edited by] G. Petur Nielsen and Andrew E. Rosenberg. Other titles: Bone.

Description: Second edition. | Salt Lake City, UT : Elsevier, Inc., [2017] | Includes bibliographical references and index.

Identifiers: ISBN 978-0-323-47777-2

Subjects: LCSH: Bones--Tumors--Handbooks, manuals, etc. | MESH: Bone Neoplasms--pathology--Atlases. | Bone Neoplasms--diagnosis--Atlases.

Classification: LCC RC280.B6 N54 2017 | NLM WZ 17 | DDC 616.8’4--dc23

International Standard Book Number: 978-0-323-47777-2

Cover Designer: Tom M. Olson, BA

Printed in Canada by Friesens, Altona, Manitoba, Canada

Last digit is the print number: 9 8 7 6 5 4 3 2 1

Dedications

To my wife and family.

GPN

To my daughters, Olivia and Miranda, who are my lifelong joy; my parents, Philip and Evelyn, who did their best; my siblings, David, Stuart, and Elaine, who have been supportive; my friend and colleague, Al, who always has my back; my teachers who have helped show me the way; my colleagues with whom I have had the honor to be in the trenches; and the patients who have given me their trust. AER

To my father, Dhirendra, and mother, Shashi.

Preface

The pathology of the skeleton is complex and is the morphologic expression of a broad spectrum of diseases, including those caused by genetic (sporadic and inherited), malformative, inflammatory, metabolic, circulatory, traumatic, iatrogenic, and neoplastic disorders. Bone tumors, including both neoplasms and various conditions that may simulate them, are the focus of our book. This topic is one of the most challenging areas in surgical pathology for several reasons: Bone tumors are uncommon, making it difficult to acquire the necessary experience with their histological variants and mimics; the correct diagnosis usually requires the careful integration of radiological imaging studies and clinical findings; the implications of a diagnosis on a patient can be life changing; and medical schools and pathology training programs often have insufficient expertise to provide medical students and young pathologists with the skills needed to diagnose these lesions accurately and precisely.

This book reflects the philosophy and high standards practiced by the truly multidisciplinary team of physicians at the Massachusetts General Hospital and University of Miami, who have diagnosed and surgically treated tens of thousands of patients with bone tumors for many decades. Also important to acknowledge are the contributions of the many fellows and residents who participated in the efforts of patient care.

The authors are subspecialized physicians who have dedicated their professional lives to the diagnosis and surgical management of bone tumors. As a result, the figures include beautiful and classic examples and unusual variants of many of the diseases discussed and are the product of painstaking correlations between the clinical, imaging, macroscopic, histological, immunohistochemical, and molecular characteristics of bone tumors. The text synthesizes the literature and our combined extensive experience, and the images have been selectively culled from the patient files of the Massachusetts General Hospital, the University of Miami Miller School of Medicine, and the private consultations of the authors. The book is constructed in a thematic format with sections representing groups of related diseases and the chapters discussing individual entities and their differential diagnosis.

Accordingly, this textbook serves as an excellent resource for medical students, residents, fellows, and practicing physicians in the disciplines of pathology, radiology, and orthopedics. Medical and radiation oncologists who treat bone tumors will also find it valuable. Our opportunity to participate in the care of patients with bone tumors has been our call and honor, and we hope to do it justice by sharing our experience with the medical community—our goal is to enhance diagnostic accuracy and to provide the biological basis for optimal treatment.

G. Petur Nielsen, MD

Pathologist, Department of Pathology

Director of Electron Microscopy

Director of Bone & Soft Tissue Pathology

Massachusetts General Hospital

Professor of Pathology

Harvard Medical School

Boston, Massachusetts

Andrew E. Rosenberg, MD

Professor and Vice Chair

Director of Bone & Soft Tissue Pathology

Department of Pathology

Miller School of Medicine

University of Miami Hospital

Miami, Florida

Acknowledgments

Text Editors

Arthur G. Gelsinger, MA

Nina I. Bennett, BA

Lisa A. Gervais, BS

Karen E. Concannon, MA, PhD

Matt W. Hoecherl, BS

Megg Morin, BA

Image Editors

Jeffrey J. Marmorstone, BS

Lisa A. M. Steadman, BS

Illustrations

Laura C. Sesto, MA

Richard Coombs, MS

Lane R. Bennion, MS

Art Direction and Design

Tom M. Olson, BA

Laura C. Sesto, MA

Lead Editor

Terry W. Ferrell, MS

Production Coordinators

Angela M. G. Terry, BA

Rebecca L. Bluth, BA

Emily C. Fassett, BA

Sections

SECTION 1: Benign Bone-Forming Tumors

SECTION 2: Malignant Bone-Forming Tumors

SECTION 3: Benign Cartilage Tumors

SECTION 4: Malignant Cartilage Tumors

SECTION 5: Fibrous and Fibrohistiocytic Tumors

SECTION 6: Fibroosseous Tumors

SECTION 7: Malignant Small Round Cell Tumors

SECTION 8: Notochordal Tumors

SECTION 9: Giant Cell-Rich Tumors

SECTION 10: Cystic Lesions of Bone

SECTION 11: Vascular Tumors

SECTION 12: Hematopoietic Tumors

SECTION 13: Miscellaneous Mesenchymal Tumors

SECTION 14: Metastatic Tumors

SECTION 15: Bone Tumor Mimics

TABLE OF CONTENTS

SECTION1:BENIGNBONE-FORMING TUMORS

4BoneIsland/Osteopoikilosis

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

10Osteoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

16OsteoidOsteoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

22Osteoblastoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION2:MALIGNANTBONE-FORMING TUMORS

32ConventionalOsteosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

52Well-DifferentiatedIntramedullaryOsteosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

58ParostealOsteosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

68PeriostealOsteosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

72High-GradeSurfaceOsteosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

76SecondaryOsteosarcoma

VikramDeshpande,MD,G.PeturNielsen,MD,and AndrewE.Rosenberg,MD

SECTION3:BENIGNCARTILAGETUMORS

82VascularCartilaginousHamartomaofChestWall

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

84Osteochondroma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

94MultipleHereditaryOsteochondromatosis

VikramDeshpande,MD,AndrewE.Rosenberg,MD,and

G.PeturNielsen,MD

98Enchondroma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

110Enchondromatosis

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

116PeriostealChondroma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

120Chondroblastoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

128ChondromyxoidFibroma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION4:MALIGNANTCARTILAGE TUMORS

138ConventionalChondrosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

150DedifferentiatedChondrosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

156ClearCellChondrosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

162MesenchymalChondrosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION5:FIBROUSAND FIBROHISTIOCYTICTUMORS

172FibrousCorticalDefect/NonossifyingFibroma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

178DesmoplasticFibroma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

184MyofibromaandMyofibromatosis

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

188Fibrosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

194BenignFibrousHistiocytoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

196SolitaryFibrousTumor/Hemangiopericytoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION6:FIBROOSSEOUSTUMORS

200FibrousDysplasia

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

212LiposclerosingMyxofibrousTumor

G.PeturNielsen,MD,AndrewE.Rosenberg,MD,and VikramDeshpande,MD

SECTION7:MALIGNANTSMALLROUND CELLTUMORS

218EwingSarcomaandRelatedTumors

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

230MelanoticNeuroectodermalTumor

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION8:NOTOCHORDALTUMORS

234Ecchordosis

VikramDeshpande,MD,AndrewE.Rosenberg,MD,and G.PeturNielsen,MD

236BenignNotochordalCellTumor

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

242Chordoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

TABLE OF CONTENTS

SECTION9:GIANTCELL-RICHTUMORS

256GiantCellTumor

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

270BrownTumor

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

274GiantCellReparativeGranuloma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION10:CYSTICLESIONSOFBONE

282IntraosseousGanglion

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

286UnicameralBoneCyst

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

294AneurysmalBoneCyst

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

306EpidermoidInclusionCyst

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION11:VASCULARTUMORS

310ConventionalHemangioma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

318Lymphangioma/Lymphangiomatosis

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

320EpithelioidHemangioma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

328PseudomyogenicHemangioendothelioma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

334EpithelioidHemangioendothelioma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

340Angiosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION12:HEMATOPOIETICTUMORS

346LangerhansCellHistiocytosis(Eosinophilic Granuloma)

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

352PrimaryLymphoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

360PlasmaCellMyeloma

VikramDeshpande,MD,G.PeturNielsen,MD,and AndrewE.Rosenberg,MD

368MastCellDisease

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

370Erdheim-ChesterDisease

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

376Rosai-DorfmanDisease

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION13:MISCELLANEOUS MESENCHYMALTUMORS

382OsteofibrousDysplasia

VikramDeshpande,MD,G.PeturNielsen,MD,and AndrewE.Rosenberg,MD

386Adamantinoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

392AdipocyticTumors

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

400Leiomyosarcoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

404Myoepithelioma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

410Schwannoma

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

414MyxopapillaryEpendymoma

VikramDeshpande,MD,G.PeturNielsen,MD,and AndrewE.Rosenberg,MD

416PhosphaturicMesenchymalTumor

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

SECTION14:METASTATICTUMORS

420MetastaticTumors

VikramDeshpande,MD,AndrewE.Rosenberg,MD,and G.PeturNielsen,MD

SECTION15:BONETUMORMIMICS

428BizarreParostealOsteochondromatous ProliferationandRelatedLesions

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

434Melorheostosis

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

438Amyloidoma

G.PeturNielsen,MD,AndrewE.Rosenberg,MD,and VikramDeshpande,MD

440GaucherDisease

G.PeturNielsen,MDandAndrewE.Rosenberg,MD

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SECOND EDITION

NIELSEN | ROSENBERG

DESHPANDE • HORNICEK • KATTAPURAM • ROSENTHAL

This page intentionally left blank

SECTION 1

Benign Bone-Forming Tumors

Bone Island/Osteopoikilosis

KEYFACTS

TERMINOLOGY

• Enostosis

CLINICALISSUES

• Incidentalradiographicfinding

• Largeboneislandsinchildrenmaybeconcerningfor osteosarcoma

• Rarelyneedtobebiopsied

• Multipleboneislandsrepresentosteopoikilosis

• Observationforsmallsolitarycharacteristiclesions,but thosethatarelargeorhaveunusualfeaturesmayrequire biopsy

IMAGING

• Mostfrequentsitesincludepelvis,proximalfemur,andribs

• Inosteopoikilosis,usuallyinvolveepiphysesofshorttubular bones

• Generallynotmuchlargerthan1cmindiameter

• Homogeneouslyradiodenselesionswithspiculated margins,whichmergewithsurroundingcancellousbone

MACROSCOPIC

• Hard,solid,tan-white

• Peripheryblendswithsurroundingcancellousbone

MICROSCOPIC

• Consistofcortical-typebonecontaininghaversian-like canals

• Predominatelylamellarbutmaybefocallywoven

• Osteopoikilosisboneislandtumorsareidenticalto sporadic,solitaryboneislands

TOPDIFFERENTIALDIAGNOSES

• Well-differentiatedosteosarcoma

• Scleroticmetastases

(Left)Specimenradiograph showsexcisedfemoralhead containingaboneisland.The boneislandisovaland radiodense.Theperipheryhas astellatemarginmergingwith theneighboringcancellous bone.(Right)Gross photographoffemoralhead showsboneislandbeneaththe articularsurface.Thebone islandisdense,tan-white,and hasaspiculatedborderasit mergeswiththeadjacentbony trabeculae.

(Left)Radiographofthepelvis demonstratesauniformly denseboneisland﬈.The spiculatedmarginsrepresent extensionsfromthelesion, whichmergewiththe surroundingcancellousbone. (Right)CTofaboneisland﬈ demonstratesthatitisofthe samedensityascorticalbone. Theabsenceofalytic componentcanbedifficultto confirmwithoutadditional cross-sectionalimaging,an importantfeatureto distinguishboneislandfrom other,moresinisterboneforminglesions.

SpecimenRadiographofBoneIsland

GrossPhotographofBoneIsland

BoneIslandInvolvingPelvis

CTScanofPelvicBoneIsland

Bone Island/Osteopoikilosis

TERMINOLOGY

Synonyms

• Enostosis

• Spottedbonedisease

Definitions

• Benignbone-formingtumorcomposedofcortical-type bonethatdevelopswithinmedullarycavity

• Osteopoikilosis

○ Syndromecharacterizedbypresenceofmultiple(usually many)boneislands

○ CanbeassociatedwithBuschke-Ollendorffsyndrome andmelorheostosis-likelesions

ETIOLOGY/PATHOGENESIS

Neoplasm

• Causeofboneislandisunknown

• Osteopoikilosismaybeinheritedinautosomaldominant fashion

○ AssociatedwithmutationsandlossoffunctioninLEMD3 locatedon12q14

CLINICALISSUES

Presentation

• Incidentalradiographicfindingandasymptomatic

○ Largeboneislandsmaybepainful

• Uncommoninchildren

• Lesionsinosteopoikilosismayundergoslow,progressive enlargementorinvolution

Treatment

• Observationforsmallsolitarylesionswithclassic radiographicfeatures

• Largervariantsorcasesinadolescentsmayrequirebiopsy toexcludemoreaggressivelesions,suchaswelldifferentiatedosteosarcomaandscleroticmetastasesin adults

Prognosis

• Excellent

• Malignanttransformationdoesnotoccur

IMAGING

GeneralFeatures

• Location

○ Mostfrequentsitesarepelvis,ribs,andproximalfemur

○ Inadults,incidenceinpelvicbones(1.0%)andribs(0.5%)

○ Epiphysealintubularbones

○ Osteopoikilosisisbilateralandsymmetricalin distributionandinmetaphysealandepiphysealregions oftubularbones

– Anybonemaybeaffected,includingtarsalandcarpal bones

• Size

○ Usually<1cmindiameter

○ Infrequently,"giant"boneislandsseveralcmindiameter occur

RadiographicFindings

• Small

• Oval ○ Longaxisofovalisusuallyparalleltomechanicalstresses onbone,representingadaptationtoWolfflaw

• Singleormultiple

• Largervariantsmayabutorbebasedonendostealsurface

○ Donotinvolvecortexanddonotelicitperiosteal reaction

• Homogeneouslyradiodenselesionswithspiculated marginsthatmergewithsurroundingcancellousbone

MRFindings

• DarkonT1WIandT2WI,similartonormalcorticalbone

CTFindings

• Smallstellatemedullarylesionwithcharacteristicsof cortical-typebone

BoneScan

• Canshowsomeuptakeofradionuclide

MACROSCOPIC

GeneralFeatures

• Hard,solid,andtan-white;peripheryblendsinto surroundingtrabeculae,whichaccountsforirregular spiculatedmargins

MICROSCOPIC

HistologicFeatures

• Consistofcortical-typebonecontaininghaversian-like canals

• Predominatelylamellarbutmaybefocallywoven

• Osteoblastsliningsurfacesareflatandquiescent

• Osteocytesaresmallandcytologicallybanal

• Boneislandsinosteopoikilosisareidenticaltosporadic, solitaryboneislands

DIFFERENTIALDIAGNOSIS

Well-DifferentiatedOsteosarcoma

• Infiltrative,composedofproliferatingmildlyatypical spindlecellsandwovenbone

ScleroticMetastases

• Usuallyinadultsandcontainhistologicallymalignantcells

DIAGNOSTICCHECKLIST

ClinicallyRelevantPathologicFeatures

• Multiplelesionsraisedifferentialdiagnosisofblastic metastases;multipleboneislandsseeninosteopoikilosis

PathologicInterpretationPearls

• Lesionisintramedullary,corticalintype,andpredominately lamellarinarchitecture

SELECTEDREFERENCES

1. KorkmazMFetal:Osteopoikilosis:reportofafamilialcaseandreviewofthe literature.RheumatolInt.ePub,2014

(Left)CTscanoftheshoulder demonstratesaverylarge boneislandſtofthescapula atthebaseofthecoracoid process.Thelesionis uniformlyandcompletely denseandabutsthecortex butdoesnotinvadeor transgressit.(Right)MRofa roundboneislandſtshows thatitisuniformly hypointenseandabutsthe cortexbutdoesnotinvadeor transgressit.Themarrow adjacenttotheossifiedlesion iscompletelynormal.

(Left)Aboneislandofthe centralportionofthe vertebralbodyshows characteristicfeatures: Uniformdensityand spiculatedmarginsſt.The lesionissurroundedby unremarkablecancellous bone,andthecortexis uninvolved.(Right)Agiant boneislandofthevertebrais shownſtalmostfillingthe entirevertebralbody.Such largelesionscanshowuptake onisotopebonescansdueto theirsize.Inotherrespects, thefeaturesaresimilarto conventionalboneislands.

(Left)SagittalCTscanshowsa largeboneislandinvolvingthe pedicleandfacetjointofT12 ſt.Thelesionfillsa significantportionofthe medullarycavityandmerges withtheoverlyingcortex.The intramedullarymarginis undulatingandfocally spiculated.Smallerlesionsare seenintheadjacentvertebra. (Right)AxialCTofalarge boneislandinvolvesthe pedicleandfacetjointofT12 ſt.Thetumorhasthesame densityasthecortex,whichis unremarkable.

CTScanofBoneIsland

MRofBoneIsland

BoneIslandofSpine

BoneIslandofSpine

BoneIslandInvolvingPedicle

BoneIslandofPedicle

(Left)APradiographofthe distalfemurdemonstratesa largeboneislandinvolvingthe metaphysis﬈.Notethe spiculatedmarginproximally. Inthisparticularlesion,the elongatedshapeofthelesion isapparent,representing adaptationtomechanical stresses.(Right)Lateral radiographofthedistalfemur demonstratesalargebone islandoftheposteriorportion oftheboneſt.Thelesion appearstobebasedonthe endostealsurfaceandextends intothemedullarycavity.

(Left)AxialCTshowsagiant boneislandofthedistalfemur ſtwithuniformdensityof compactboneandspiculated margins.Thebroadbaseof thetumorisattachedtothe endostealsurfaceofthe posteriorcortex.(Right) Isotopebonescanshowsa smallamountofuptakeſtin thelateralaspectoftheright proximalhumerus.Asmall amountofuptakecanbe presentinboneislands becausetheyareactivelybone forming.Itshouldnotbe consideredamarkerof malignancy.

(Left)CTscandemonstratesa largeboneislandofthe proximalhumerusſt.The lesioniseccentric,abutsthe endostealsurfaceofthe cortex,andextendsinthe medullarycavityinan irregularfashion.(Right) CoronalT1-weightedMRof theboneislandofthe humerusdemonstratesthe homogeneouslowsignal intensityofthelesion﬈.The boneislandisbasedonthe innersurfaceofthecortexand hasirregularmargins.The adjacentmarrowis unremarkable.

LargeBoneIslandofFemur

LargeBoneIslandofFemur

CTofBoneIslandofFemur

BoneScan

LargeBoneIslandofProximalHumerus MRofBoneIslandofHumerus

(Left)APradiograph demonstratesosteopoikilosis withmultipleboneislands involvingtheendsoftheshort tubularbones.Thesmall lesionsclusterattheendsof thebones.(Right)AP radiographofthekneesshows thetypicalfeaturesof osteopoikilosis.Alarge numberofsmallboneislands aresymmetricallydistributed inaprimarilyjuxtaarticular andmetaphysealdistribution. Despitetheirmultiplicity,each lesionindividuallyhasthe featurestypicalofabone island.

(Left)ReformattedcoronalCT scanofthehipshowsmultiple smallboneislandsthatare juxtaarticularand metaphysealindistribution. Eachlesionisindividually indistinguishablefroma solitaryboneisland.(Right) T1-weightedMRofthekneein apatientwithosteopoikilosis demonstratesthatthebone islandsaresmall,rather uniforminsize,ovalinshape, anduniformlydense.Each lesionisindividually indistinguishablefroma solitaryboneisland.

(Left)CoronalCT demonstratesan intramedullaryboneisland involvingtherightmandible ſt.Thelesionisscleroticwith thesamedensityasthe surroundingcortex.(Right) Grossphotographshowsrib andadjacentcostalcartilage. Anelongate,dense,andtanwhiteboneislandfillsthe involvedsegmentofthe medullarycavityſt.The adjacentcortexandcostal cartilageareunremarkable.

Osteopoikilosis

MRofOsteopoikilosis

BoneIslandofMandible

GrossPhotoofBoneIslandArisinginRib

GrossPhotograph

Whole-MountSection

LightMicroscopy LightMicroscopy

(Left)Grossphotographshows anexcisedfemoralheadwith anincidentalboneisland﬈. Thetumorislocatedbeneath thearticularcartilageand mergeswiththesurrounding trabecularbone.The neighboringmarrowisfatty andunremarkable.(Right) Low-powerviewshowsabone islandinvolvingthemedullary cavityoftheproximalfemur ﬈.Thelesioniscomposedof cortical-typebonethatblends imperceptivelywiththe surroundingtrabecularbone.

(Left)Histologicsectionofa solitaryboneislandisshown. Theboneislandiscomposed ofcortical-typebonewith haversian-likecanalsand transitionsintotheadjacent cancellousbone.The surroundingmarrowis predominatelyfattywith scatteredislandsof hematopoieticcells.(Right) Boneislandwithnumerous haversian-likesystems scatteredthroughoutthe lesionisshown.Theboneof thetumorissharply demarcatedfromtheadjacent marrow.

ofBoneIsland

(Left)Closeviewofthe transitionbetweenthe peripheryoftheboneislandis shownasitmergeswithan adjacentbonetrabeculum. Theboneiswovenand lamellarandhasasharp borderwiththesurrounding fattyandhematopoietic marrow.(Right)Resected femoralheadfromapatient withosteopoikilosisshows thatnumerousboneislands arepresentwithinthe medullarycavity.Thelesions areroundtoovalandvaryin size.Themarginsareirregular andspiculated.

Periphery

Osteopoikilosis

Osteoma

TERMINOLOGY

• Benignsurfacebone-formingtumor,usuallycomposedof cortical-typebone

CLINICALISSUES

• Usuallysmallandsolitary

• Commonlyasymptomaticandincidentalfinding

• Mostfrequentlydevelopincraniofacialskeleton

• Appendiculartumorsareveryuncommon

• MultiplelesionsraisepossibilityofGardnersyndrome

• Asymptomaticlesionscanbeobserved

• Symptomaticlesionscanbeconservativelyexcised

IMAGING

• Smallanduniformlyradiodense

• Sharplymarginatedwithwell-formedperiostealreaction

• Ovaltodome-shapedwithbroadattachmenttocortical surface

• Underlyingcortexisnotinvolved

MACROSCOPIC

• Generally<2cmindiameter;round,tan-white,andhard

• Resemblescorticalbonewithwhichitmerges

MICROSCOPIC

• Consistsmainlyoflamellarboneadmixedwithsomewoven bone

• Bonehascortical-typearchitecture

• Minorityofosteomascomposedoftrabecularbone

• Lesionalosteoblastsandosteocytesusuallyinconspicuous

DIAGNOSTICCHECKLIST

• Well-formedcorticalboneandbanalcytologydistinguishes osteomafromosteosarcoma

• Intactcortexandabsenceofcartilageexcludes osteochondroma

• Hypocellularityoflesionisevidenceagainstmyositis ossificans

• Melorheostosisandosteomaarehistologicallysimilar

OsteomaofFibula

OsteomaofLongBoneResectionSpecimen

Radiographofproximallowerlegshowsadense,welldefinedossificmassonthesurfaceoftheproximalfibula. Thereisthick,periostealboneappositionproximaltothe massthatistriangularinshape.

Osteomaiscomposedofhard,dense,compactbonewitha broadattachmenttotheunderlyingcortex.Atriangularshapedzoneofsubperiostealboneispresentproximaland distaltotheosteoma.

Osteoma

TERMINOLOGY

Abbreviations

• Osteoma(OS)

Synonyms

• Toruspalatinus(palate)andmandibularis(mandible)

Definitions

• Benignsurfacetumorcomposedofcortical-typebone

○ Minoritycomposedoftrabecularbone(trabecular osteoma)

CLINICALISSUES

Epidemiology

• Incidence

○ Paranasalsinusosteoma:3-4%

○ Cranialosteomaatautopsy:4-5%

○ Accountsfor0.03%ofbiopsiedprimarybonetumors

• Age

○ Mostcommonin4thto6thdecadesoflife

• Sex

○ Nopredilection

Site

• Craniofacialskeletonmostcommon

○ Oftenlocatedinfrontalandethmoidsinuses(75%)

○ Sphenoidsinus,cranium,jaw

• Appendicularskeletonrare

○ Longtubularbones

– Femurandtibiamostcommon

Presentation

• Slow-growingsmalllesions;usuallyincidentalfinding

• Largelesions:Symptomsrelatedtoanatomiclocation

○ Sinustumors:Obstructionandmucocele

○ Orbitaltumors:Exophthalmosandvisiondisturbances

○ Oraltumors:Interferewithdenturesandmastication

○ Appendiculartumors:Palpablehardmass

• Usuallysolitary

○ MultipletumorsmaybeseeninGardnersyndrome

Treatment

• Observation

• Simpleexcision

Prognosis

• Excellent,norecurrence

IMAGING

GeneralFeatures

• Uniformlyradiodensesurfacelesionwelldemarcatedfrom softtissue

• Ovoidwithbroadbaseofattachmenttocortex

• Denseperiostealreactionalongmarginofattachmentmay bepresent

MRFindings

• LowsignalintensityonT1-andT2-weightedimages

○ Lesiondoesnotenhancewithcontrast

CTFindings

• Well-delineatedsurfacemasswithcorticaldensity

BoneScan

• Mayshowincreasedornoradiotraceruptake

MACROSCOPIC

GeneralFeatures

• Generally<2cmindiameter

• Oval,round,orhemispheric

• Hard

• Tantowhite

• Resemblescorticalbonewithwhichitmerges

• Well-formed,triangular-shapedsubperiostealreactivebone maysurroundattachmentsitetocortex

MICROSCOPIC

HistologicFeatures

• Admixtureoflamellarandwovenbonewithhaversian-like systems

• Infrequentlycomposedoftrabecularbone

• Growinglesionmayhavefibrouscomponentmimicking fibroosseoustumor

• Osteoblastsrimmingboneareinconspicuousandelongate

○ Growinglesionslinedbyplumpmetabolicallyactive osteoblasts

– Abundanteosinophiliccytoplasmandnucleipolarized awayfrombone-formingsurface

• Inactiveosteoblastsandosteocyteshavesmallrounddark nucleiandnonucleoli

DIFFERENTIALDIAGNOSIS

Bone-FormingLesions

• Parostealosteosarcoma

○ Containsprominentspindlecellcomponent

• Juxtacorticalmyositisossificans

○ Composedofhypercellularcancellousbone

• Melorheostosis

○ Drippingcandlewaxconfiguration

• Osteochondroma

○ Hascartilagecap

DIAGNOSTICCHECKLIST

PathologicInterpretationPearls

• Well-formedcorticalboneandbanalcytologydistinguishes osteomafromosteosarcoma

• Intactcortexandabsenceofcartilageexcludes osteochondroma

• Hypocellularityisevidenceagainstmyositisossificans

• Melorheostosisandosteomaarehistologicallysimilar

SELECTEDREFERENCES

1. HalawiAMetal:Craniofacialosteoma:clinicalpresentationandpatternsof growth.AmJRhinolAllergy.27(2):128-33,2013

2. GreenspanA.Benignbone-forminglesions:osteomaetal:clinical,imaging, pathologic,anddifferentialconsiderations.SkeletalRadiol.22(7):485-500, 1993

3. O'ConnellJXetal:Solitaryosteomaofalongbone.Acasereport.JBone JointSurgAm.75(12):1830-4,1993