Imaging of Soft Tissue Tumors

Springer-Verlag Berlin Heidelberg GmbH

A. M. De Schepper (Editor)

P. M. Parizel 1. De BeuckeleerF. Vanhoenacker (Coeditors)

Imaging ofSoftTissue Tumors

Second Editionwith 354 Figures in 955 Separate Illustrationsand 32 Tables

Springer

Arthur M. De Schepper, MD, PhD - Professor and ChairmanPaul M. Parizel, MD, PhD - Staff RadiologistLuc De Beuckeleer, MD - RadiologistFilip Vanhoenacker, MD - Staff Radiologist

Department of Radiology, University Hospital of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Library of Congress Cataloging-in-Publication DataImaging of soft tissue tumors/A.M. De Schepper, editor; P.M. Parizel . . . [et a1. J,coeditors. -2nd ed.p.cm. Includes bibliographical references and index.I. Soft tissue tumors-Imaging. I. Schepper, A. M. De, 1937-. II. Parizel, P.M.(Paul M.), 1956-. RC280.S66143 2001 616.99'40754-dc21 2001020039

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Preface to the Second Edition

At the time of writing, our group has had more than 10years' experi­ence in the imaging of soft tissue tumors. We are now, - more thanever, - convinced that a multidisciplinary dialogue between orthope­dic surgeons, oncologists, pathologists and radiologists is imperativefor the medical management of these lesions. The common goals of allspecialists dealing with soft tissue tumors should be: early detection,minimally invasive staging and grading procedures, specific diagnosis(or suitably ordered differential diagnosis), guided percutaneous biop­sies, and the most suitable therapy. This approach will guarantee thepatient the optimal chances of survival with the best possible quality oflife. To help us achieve these goals, we have established a Commissionfor Bone and Soft Tissue Tumors at the University Hospital in Antwerp,which convenes every 2 weeks. This multidisciplinary group formu­lates opinions and recommendations on diagnosis, prognosis, treat­ment and follow-up, and is highly valued by referring physicians. Inaddition, we are organizing a Belgian Registry of Soft Tissue Tumorswith the cooperation of all Belgian centers in which MRI equipment isavailable and intend to invite students and investigators from all overthe world to share our scientific interest in this fascinating field of me­dical imaging.

The main objective of this second edition of "Imagingof Soft TissueTumors"is to provide radiologists with an updated and easy-to- read refe­rence work. This second edition includes new literature references and il­lustrations. Older illustrations have been replaced with higher qualityimages, generated by newer equipment and/or MRI pulse sequences.New tables organizing information into summaries have been includedand the subject index has been updated. Most importantly, the text con­tains newer insights (for instance about fibrohistiocytic tumors), and re­flects our own experience of increasing understanding of soft tissue tu­mors and their imaging.The chapter about magnetic resonance imaginghas been shortened, and now focuses mainly on principles, pulse se­quences and applications that are directly related to the examination ofsoft tissues and soft tissue tumors. We have included new chapters on"Soft Tissue Tumors in Pediatric Patients" and"Soft Tissue Lymphoma",and also a chapter on the controversial subject of (percutaneous) biopsy.

The readers and the reviewers of our book will judge whether wehave succeeded in our objectives.

Finally, we would like to thank our editor and Mrs. Mennecke­Buhler at Springer-Verlag for sharing in the challenge of editing a se­cond edition of this book on a rare pathology.

Antwerp, July 2001 Arthur M. De Schepper

Preface to the First Edition

Although the soft tissues constitute a large part of the human body, softtissue tumors are rare, accounting for less than 1% of all neoplasms.The annual incidence of benign soft tissue tumors in a hospital popula­tion is 300 per 100000. Moreover,benign lesions outnumber their ma­lignant counterparts by about 100 to 1. The clinical and biochemicalfindings of soft tissue tumors are frequently nonspecific. The first signis usually a soft tissue swelling or a palpable mass with or without painor tenderness. Laboratory results are frequently normal or show mini­mal nonspecific changes.

Until a few decades ago, detection of soft tissue tumors usually didnot take place until late in the course of disease. This resulted fromtheir low incidence and nonspecific clinical findings and from the poorsensitivity of conventional radiography, which was the only imagingtechnique available.Soft tissue tumors and soft tissue disorders in gen­eral were practically unknown to radiologists until the introductionof ultrasound and computed tomography (CT). Unfortunately, thesemethods suffered from inherent drawbacks, such as the poor specif­icity of ultrasound and the poor contrast resolution of CT.

Many of these problems were solved by the introduction ofmagnetic resonance imaging (MRI). Thanks to its high contrast tis­sue resolution and its multiplanar imaging capability, new horizonswere opened for imaging soft tissues. Today, a correct assessment ofdisorders of bones, joints, or soft tissues is unimaginable withoutMRI.

In view of recent developments in surgery, radiation therapy, sys­temic chemotherapy, and regional perfusion techniques, the imagingof soft tissue tumors is gaining in importance. Correct diagnosis in­cludes the detection, characterization, and staging of the lesions. Theinadequate diagnosis and therapy of soft tissue sarcomas frequentlyresults in tumor recurrence, necessitating major therapeutic "aggres­sion." MRI is the optimal imaging technique for avoiding inadequateassessment.

Despite the interest of many groups of radiologists in the subjectand despite the considerable number of overview articles that havebeen published in the radiologic literature, soft tissue tumors receiveonly minimal attention in modern state-of-the-art books on musculo­skeletal imaging . Nevertheless, since all radiologists involved in the fas­cinating field of MRI are now confronted with tumoral pathology ofsoft tissues, there is a need for an illustrated radiologic guide on thesubject.

From the beginning of our experience using MRI, back in 1985,wehave been interested in soft tissue tumors. Our initial findings were

VIII Preface to the FirstEdition

discussed at an international congress in 1992.Conflicting findings inthe literature concerning the sensitivity and specificity of MRI, whichwere mainly caused by the limited number of patients in published se­ries, prompted us to start a multicenter European study. At the Europe­an Congress of Radiology 1993 in Vienna, 29 co-investigators from allover Europe agreed to participate (see the list 'Investigators of Multi­centric European Study on Magnetic Resonance Imaging of Soft TissueTumors'). More than 1000cases were collected, which constitute the ba­sis of the radiologic work we prepared.

It was not our intention to write the 'all you ever wanted to know'book on soft tissue tumors. This objective has already been achievedfor the pathology of soft tissue tumors by Enzinger and Weiss. Al­though their famous textbook contains a brief discussion of modernmedical imaging, you will find it rarely on the office desk of radiolo ­gists. This present book is intended to serve as a reference guide forpractising radiologists and clinicians seeking the optimal imaging ap­proach for their patients with a soft tissue tumor.

The book is divided into four sections. In the first section we discussthe different imaging modalities and their respective contribution tothe diagnosis of soft tissue tumors. As MRI is generally accepted to bethe method of choice, there is a detailed theoretical description of thistechnique combined with a short discussion of imaging sequences. Wealso included a chapter on scintigraphy of soft tissue tumors, in whichthe current literature on the subject is summarized because scintigra­phy was hardly used in our own patient material.

The second part deals with staging and characterization of softtissue tumors and is concluded by a chapter on general imaging strat­egy. Tumor-specific imaging strategy is, where needed, added at theend of the tumor-specific chapters, which are collected in Part III.These chapters include a short description of epidemiology, clinicaland pathological presentation, and a detailed discussion of imagingfindings . For this Part , we used the classification of E. B. Chung (Cur­rent classification of soft tissue tumors. In: Fletcher CD, McKee PH(eds) Pathobiology of soft tissue tumors, 1st edn. Churchill Living­stone, Edinburgh, 1990,pp 43-81), which is an updated version of themost comprehensive system of classification, that of the World HealthOrganization. Because the illustrations originate from differentinstitutions using different MR systems and pulse sequences, thefigure legends only mention the plane of imaging (sagittal, axial,coronal), the kind of sequence (SE,TSE, GRE,.. .), and the weighting(TI, T2).

The fourth part consists of only one chapter dealing with post -treat­ment imaging findings .

I would like to thank my co-editors Dr. Paul Parizel, Dr. Frank Ra­mon, Dr. Luc De Beuckeleer, and Dr. Jan Vandevenne, and all the co­authors for the tremendous job they have done . From this work Ilearned that writing a good book requires a sabaticcal leave, whichgood fortune I did not have.

As previously mentioned, it has been possible to include many of theillustrations shown in the book only because of the cooperation of the29 European investigators, to whom lowe my gratitude. We gratefullyacknowledge the support of Prof. Eric Van Marek, pathologist at our in-

Preface to the FirstEdition IX

stitution, for reviewing the manuscript, and of Ingrid Van der Heyden(secretary) for her aid in preparing so many chapters.

Finally, I wish to express my gratitude to Springer-Verlag and toDr. Ute Heilmann for sharing the challenge of preparing this book withus.

Antwerp, June 1996 Arthur M. De Schepper

Contents

Imaging Modalities .

1 Ultrasound of Soft Tissue TumorsR. Ceulemans and M. van Holsbeeck

2 Color Doppler UltrasonographyH.-J. van der Woude, K.1. Verstraete, and J.1. Bloem

3

21

3 Plain Radiography, Angiography, and Computed TomographyA. M. Davies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31

4 Nuclear Medicine ImagingP.P.Blockx and 1. Carp . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45

5 Magnetic Resonance ImagingH. Bosmans, P. Brys, and G. Marchal 61

6 Dynamic Contrast-enhanced Magnetic Resonance ImagingK.L.Verstraete and H.-J. van der Woude 83

Staging, Characterization, and Grading . . . . . . . . . . . . . . . . . . . .. 105

7 StagingS.M. Levine, R. M. Terek, T.J.Hough, and G.A. Tung 107

8 Grading and Characterization of Soft Tissue TumorsA.M. De Schepper 123

9 General Imaging StrategyF.Ramon 143

Imaging ofSoftTissueTumors 147

10 Tumors of Fibrous TissueA.M. De Schepper and J. E.Vandevenne .. . . . . . . . . . . . . . . . . . 149

11 Fibrohistiocytic TumorsL.De Beuckeleer 181

XII Contents

12 Lipomatous TumorsM. C.Marques, H. Garcia, and F. Vanhoenacker 195

13 Tumors and Tumorlike Lesions of Blood VesselsF. Ramon 225

14 Lymphatic TumorsL. van den Hauwe and F. Ramo n . . . . . . . . . . . . . . . . . . . . . . . . . . 245

15 Tumors of Muscular OriginP. C.Seynaeve, L. L. Mortelmans, and A. M. De Schepper . . .. . 255

16 Synov ial Tumo rsF. Vanhoenacker, J.W. M.Van Goethem, J. E.Vandeve nne,and M. Shahabpo ur 273

17 Tumors of Peri ph eral NervesP. M. Parizel, W. A. Simoens, C.Mato s, and K. L.Verstraete ... 301

18 Extraskeletal Cartilaginous and Osseo us TumorsH. R. Degryse and F. Aparisi 331

19 Primitive Neuroectodermal Tumors and Related LesionsW. A.Simoens and H. R. Degryse . . . . . . . . . . . . . . . . . . . . . . . .. 353

20 Lesions of Uncertain OriginH. R. Degryse 363

21 Pseudotumoral LesionsR.Salgado, J. Alexiou, and J.-L. Engelholm . . . . . . . . . . . . . . . .. 387

22 Soft Tissue Metas tasi sJ. Alexiou, J. -L. Engelho lm, and L. De Beuckeleer . . . . . . . . . . . 413

23 Soft Tissue LymphomaP. Bracke, F. Vanhoenacker, and A. M. De Schep per 427

24 Soft Tissue Tumors in Pediatric PatientsA.M.De Schepper, L. De Beuckeleer,and J.E.Vandevenne . . . . 433

25 Biopsy of Soft Tissue TumorsA. M. De Schepper 447

Imaging AfterTreatment 453

26 MRI in the Follow-up of Malignantand Aggressive Soft Tissue TumorsD.Vanel, L. G. Shapeero, J.M. Guinebretiere, A. Lecesne,and J. Genin 455

Subjec t Ind ex 463

Contributors

Jean Alexiou, MDDepartment of Radiology, Institut BordetRue Heger-Border 1,1000 Brussels, Belgium

Francesco Aparisi, MDDepartment of Magnetic Resonance ImagingClinica Virgen del ConsueloCallosa de Ensarria 12,46007 Valencia, Spain

Pierre P.Blockx, MDDepartment of Nuclear Medicine, Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

[ohan 1. Bloem, MD,PhDDepartment of Diagnostic Radiology and Nuclear MedicineLeiden University Hospital, Gebouw 1,C2-SPB 9600,2300 RC Leiden, The Netherlands

Hilde Bosmans, MD,PhDDepartment of Radiology, Universitair Ziekenhuizen LeuvenKatholieke Universiteit LeuvenHerestraat 49, 3000 Leuven, Belgium

Peter Bracke, MDDepartment of Radiology, KLINAAugustijnslei 100,2930 Brasschaat, Belgium

Peter Brys, MDDepartment of Radiology, Universitair Ziekenhuizen LeuvenKatholieke Universiteit LeuvenHerestraat 49,3000 Leuven, Belgium

Laurens Carp, MDDepartment of Nuclear Medicine, Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Ruth Ceulemans, MDDepartment of Diagnostic Radiology and Nuclear MedicineLeiden University Hospital, Gebouw 1,C2-SPB 9600,2300 RC Leiden, The Netherlands

XIV Contributors

A. Mark Davies, MB, ChB, DMRD, FRCRMRI Centre, Royal Orthopaedic HospitalThe Woodlands, Bristol Road SouthBirmingham B 31 2AP,UK

Luc De Beuckeleer, MDDepartment of Radiology, Sint Augustinus Ziekenhuis, WilrijkUniversitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Arthur M. De Schepper, MD, PhDDepartment of Radiology, Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Hendrik R. Degryse, MDDepartment of Radiology, KLINA,Brasschaat,Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Iean-Luc Engelholm,MDDepartment of Radiology, Institut BordetRue Heger-Border 1,1000 Brussels, Belgium

Helena Garcia, MDDepartment of Pathology, Hospitais da Universidade de CoimbraPrac. Prof. Mota Pinto, 3000 Coimbra, Portugal

J.Genin,MDDepartment of Surgery, Institut Gustave-RoussyRue Camille Desmoulins, 94805 Villejuif, France

J.M. Guinebretiere, MDDepartment of Pathology, Institut Gustave-RoussyRue Camille Desmoulins, 94805 Villejuif, France

Timothy J. Hough, MDDepartment of Diagnostic Imaging, Rhode Island HospitalBrown University School of Medicine593 Eddy Street, Providence, RI 02093, USA

A. Lecesne, MDDepartment of Radiology, Institut Gustave-RoussyRue Camille Desmoulins, 94805 Villejuif, France

Scott M. Levine, MDDepartment of Diagnostic Imaging, Rhode Island HospitalBrown University School of Medicine593 Eddy Street, Providence, RI 02093, USA

Contributors XV

Guy Marchal, MD, PhDDepartment of Radiology, Universitair Ziekenhuizen LeuvenKatholieke Universiteit LeuvenHerestraat 49, 3000 Leuven, Belgium

M. Cristina Marques, MDDepartment of Radiology, Hospitais da Universidade de CoimbraPrac. Prof. Mota Pinto, 3000 Coimbra, Portugal

Celso Matos , MDDepartment of Radiology, Hopital ErasmeUniversite Libre de BruxellesRoute de Lennik 808,1070 Brussels, Belgium

Lieven 1. Mortelmans, MDDepartment of Diagnostic RadiologyAlgemeen Ziekenhuis MiddelheimLindendreef 1,2020 Antwerp, Belgium

Paul M. Parizel, MD, PhDDepartment of Radiology, Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Frank Ramon, MDDepartment of Radiology, Algemeen Ziekenhuis Maria MiddelaresHospitaalstraat 17,9100 St.-Niklaas, Belgium

Rodrigo Salgado, MDDepartment of Radiology, Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Patrick C.Seynaeve, MDDepartment of Diagnostic Radiology, MR Unit CAZK GroeningheLoofstraat 43, 8500 Kortrijk, Belgium

Maryam Shahabpour, MDDepartment of Diagnostic RadiologyAcademisch Ziekenhuis Vrije Universiteit BrusselLaarbeeklaan 101,1090 Brussels, Belgium

L.G. Shapeero, MDDepartment of RadiologyUniformed Services University of the Health SciencesBethesda

William A. Simoens, MDDepartment of Radiology, Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilri jkstraat 10,2650 Edegem, Belgium

XVI Contributors

Richard M. Terek, MDDepartment of Orthopedic Surgery, Rhode Island HospitalBrown University School of Medicine593 Eddy Street, Providence, RI 02093, USA

Glenn A. Tung, MDDepartment of Diagnostic Imaging, Rhode Island HospitalBrown University School of Medicine593 Eddy Street, Providence, RI 02093, USA

Luc van den Hauwe, MDDepartment of Radiology,KLINA,Brasschaat,Universitair Ziekenhuis AntwerpenUniversity of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Henk-Jan van der Woude, MD, PhDDepartment of Diagnostic Radiology and Nuclear MedicineLeiden University Hospital, Gebouw 1,C2-SPB 9600,2300 RC Leiden, The Netherlands

Iohan W.M.Van Goethem, MD, PhDDepartment of Radiology,Algemeen Ziekenhuis Maria Middelares, St NiklaasUniversitair Ziekenhuis Antwerpen, University of AntwerpWilrijkstraat 10,2650 Edegem, Belgium

Marnix van Holsbeeck, MDDepartment of Diagnostic RadiologySection Musculoskeletal Radiology and Emergency RadiologyHenry Ford Hospital2799 West Grand Boulevard, Detroit, MI 48202-2689, USA

Jan E.Vandevenne, MDDepartment of Radiology, St.-Jan GenkSchiepse Bos 6,3600 Genk, Belgium

Daniel Vanel, MDDepartment of Radiology, Institut Gustave- RoussyRue Camille Desmoulins, 94805 Villejuif, France

F.Vanhoenacker, MDDepartment of Radiology, Algemeen Ziekenhuis S1. Maarten,Duffel-Mechelen, Department of Radiology,Universitair Ziekenhuis Antwerpen, University of Antwerp,Wilrijkstraat 10,2650 Edegem, Belgium

Koenraad L. Verstraete, MD, PhDDepartment of Radiology, Universitair Ziekenhuis GentUniversity of GhentDe Pintelaan 185,9000 Ghent, Belgium