Pathology and Genetics of Tumours of Soft Tissue and Bone
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bb5_1.qxd 13.9.2006 14:05 Page 3 World Health Organization Classification of Tumours WHO OMS International Agency for Research on Cancer (IARC) Pathology and Genetics of Tumours of Soft Tissue and Bone Edited by Christopher D.M. Fletcher K. Krishnan Unni Fredrik Mertens IARCPress Lyon, 2002 bb5_1.qxd 13.9.2006 14:05 Page 4 World Health Organization Classification of Tumours Series Editors Paul Kleihues, M.D. Leslie H. Sobin, M.D. Pathology and Genetics of Tumours of Soft Tissue and Bone Editors Christopher D.M. Fletcher, M.D. K. Krishnan Unni, M.D. Fredrik Mertens, M.D. Coordinating Editor Wojciech Biernat, M.D. Layout Lauren A. Hunter Illustrations Lauren A. Hunter Georges Mollon Printed by LIPS 69009 Lyon, France Publisher IARCPress International Agency for Research on Cancer (IARC) 69008 Lyon, France bb5_1.qxd 13.9.2006 14:05 Page 5 This volume was produced in collaboration with the International Academy of Pathology (IAP) The WHO Classification of Tumours of Soft Tissue and Bone presented in this book reflects the views of a Working Group that convened for an Editorial and Consensus Conference in Lyon, France, April 24-28, 2002. Members of the Working Group are indicated in the List of Contributors on page 369. bb5_1.qxd 22.9.2006 9:03 Page 6 Published by IARC Press, International Agency for Research on Cancer, 150 cours Albert Thomas, F-69008 Lyon, France © International Agency for Research on Cancer, 2002, reprinted 2006 Publications of the World Health Organization enjoy copyright protection in accordance with the provisions of Protocol 2 of the Universal Copyright Convention. All rights reserved. The International Agency for Research on Cancer welcomes requests for permission to reproduce or translate its publications, in part or in full. Requests for permission to reproduce figures or charts from this publication should be directed to the respective contributor (see section Source of Charts and Photographs). The designations used and the presentation of the material in this publication do not imply the expression of any opinion whatsoever on the part of the Secretariat of the World Health Organization concerning the legal status of any country, territory, city, or area or of its authorities, or concerning the delimitation of its frontiers or boundaries. The mention of specific companies or of certain manufacturers' products does not imply that they are endorsed or recommended by the World Health Organization in preference to others of a similar nature that are not mentioned. Errors and omissions excepted, the names of proprietary products are distinguished by initial capital letters. The authors alone are responsible for the views expressed in this publication. Enquiries should be addressed to the Communications Unit, International Agency for Research on Cancer, 69008 Lyon, France, which will provide the latest information on any changes made to the text and plans for new editions. Format for bibliographic citations: Fletcher C.D.M., Unni K.K., Mertens F. (Eds.): World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press: Lyon 2002 IARC Library Cataloguing in Publication Data Pathology and genetics of tumours of soft tissue and bone / editors, C.D.M. Fletcher ... [et al.] (World Health Organization classification of tumours ; 4) 1. Bone Neoplasms 2. Genetics 3. Pathology 4. Soft Tissue Neoplasms I. Fletcher, C.D.M. II. Series ISBN 92 832 2413 2 (NLM Classification: W1) bb5_1.qxd 13.9.2006 14:05 Page 7 Contents SOFT TISSUE TUMOURS Myxoinflammatory fibroblastic sarcoma 96 Infantile fibrosarcoma 98 WHO Classification of Soft Tissue Tumours 9 Adult fibrosarcoma 100 Myxofibrosarcoma 102 1 Adipocytic tumours 19 Low grade fibromyxoid sarcoma 104 Lipoma 20 Sclerosing epithelioid fibrosarcoma 106 Lipomatosis 23 Lipomatosis of nerve 24 3 So-called fibrohistiocytic tumours 109 Lipoblastoma / Lipoblastomatosis 26 Giant cell tumour of tendon sheath 110 Angiolipoma 28 Diffuse-type giant cell tumour 112 Myolipoma of soft tissue 29 Deep benign fibrous histiocytoma 114 Chondroid lipoma 30 Plexiform fibrohistiocytic tumour 116 Spindle cell lipoma / Giant cell tumour of soft tissue 118 Pleomorphic lipoma 31 Pleomorphic malignant fibrous histiocytoma / Hibernoma 33 Undifferentiated high grade Atypical lipomatous tumour / pleomorphic sarcoma 120 Well differentiated liposarcoma 35 Giant cell malignant fibrous histiocytoma / Dedifferentiated liposarcoma 38 Undifferentiated pleomorphic sarcoma Myxoid liposarcoma 40 with giant cells 123 Pleomorphic liposarcoma 44 Inflammatory malignant fibrous histiocytoma / Mixed-type liposarcoma 46 Undifferentiated pleomorphic sarcoma with prominent inflammation 125 2 Fibroblastic / Myofibroblastic tumours 47 Nodular fasciitis 48 4 Smooth muscle tumours 127 Proliferative fasciitis and proliferative myositis 50 Angioleiomyoma 128 Myositis ossificans and Leiomyoma of deep soft tissue 130 fibroosseous pseudotumour of digits 52 Leiomyosarcoma 131 Ischaemic fasciitis 55 Elastofibroma 56 5 Pericytic (perivascular) tumours 135 Fibrous hamartoma of infancy 58 Glomus tumours 136 Myofibroma / Myofibromatosis 59 Myopericytoma 138 Fibromatosis colli 61 Juvenile hyaline fibromatosis 63 6 Skeletal muscle tumours 141 Inclusion body fibromatosis 64 Rhabdomyoma 142 Fibroma of tendon sheath 66 Embryonal rhabdomyosarcoma 146 Desmoplastic fibroblastoma 67 Alveolar rhabdomyosarcoma 150 Mammary-type myofibroblastoma 68 Pleomorphic rhabdomyosarcoma 153 Calcifying aponeurotic fibroma 69 Angiomyofibroblastoma 71 7 Vascular tumours 155 Cellular angiofibroma 73 Haemangiomas 156 Nuchal-type fibroma 75 Epithelioid haemangioma 159 Gardner fibroma 76 Angiomatosis 161 Calcifying fibrous tumour 77 Lymphangioma 162 Giant cell angiofibroma 79 Kaposiform haemangioendothelioma 163 Superficial fibromatoses 81 Retiform haemangioendothelioma 165 Desmoid-type fibromatoses 83 Papillary intralymphatic angioendothelioma 167 Lipofibromatosis 85 Composite haemangioendothelioma 168 Extrapleural solitary fibrous tumour and Kaposi sarcoma 170 haemangiopericytoma 86 Other intermediate vascular neoplasms 173 Inflammatory myofibroblastic tumour 91 Epithelioid haemangioendothelioma 173 Low grade myofibroblastic sarcoma 94 Angiosarcoma of soft tissue 175 bb5_1.qxd 13.9.2006 14:05 Page 8 8 Chondro-osseous tumours 179 13 Fibrohistiocytic tumours 291 Soft tissue chondroma 180 Benign fibrous histiocytoma of bone 292 Extraskeletal osteosarcoma 182 Malignant fibrous histiocytoma of bone 294 9 Tumours of uncertain differentiation 185 14 Ewing sarcoma / Primitive Intramuscular myxoma 186 neuroectodermal tumour 297 Juxta-articular myxoma 188 Ewing tumour / PNET 298 Deep ‘aggressive’ angiomyxoma 189 Pleomorphic hyalinizing angiectatic 15 Haematopoietic tumours 301 tumour of soft parts 191 Plasma cell myeloma 302 Ectopic hamartomatous thymoma 192 Malignant lymphoma 306 Angiomatoid fibrous histiocytoma 194 Ossifying fibromyxoid tumour 196 16 Giant cell tumours 309 Mixed tumour / Myoepithelioma / Parachordoma 198 Giant cell tumour 310 Synovial sarcoma 200 Malignancy in giant cell tumour 313 Epithelioid sarcoma 205 Alveolar soft part sarcoma 208 17 Notochordal tumours 315 Clear cell sarcoma of soft tissue 211 Chordoma 316 Extraskeletal myxoid chondrosarcoma 213 Malignant mesenchymoma 215 18 Vascular tumours 319 Desmoplastic small round cell tumour 216 Haemangioma and related lesions 320 Extrarenal rhabdoid tumour 219 Angiosarcoma 322 Neoplasms with perivascular epithelioid cell differentiation (PEComas) 221 19 Myogenic, lipogenic, neural, Intimal sarcoma 223 and epithelial tumours 325 Leiomyoma of bone 326 Leiomyosarcoma of bone 327 BONE TUMOURS Lipoma of bone 328 Liposarcoma of bone 330 WHO Classification of Bone Tumours 225 Schwannoma 331 Adamantinoma 332 10 Cartilage tumours 233 Metastases involving bone 334 Osteochondroma 234 Chondromas 237 20 Tumours of undefined neoplastic nature 337 Chondroblastoma 241 Aneurysmal bone cyst 338 Chondromyxoid fibroma 243 Simple bone cyst 340 Synovial chondromatosis 246 Fibrous dysplasia 341 Chondrosarcoma 247 Osteofibrous dysplasia 343 Dedifferentiated chondrosarcoma 252 Langerhans cell histiocytosis 345 Mesenchymal chondrosarcoma 255 Erdheim-Chester disease 347 Clear cell chondrosarcoma 257 Chest wall hamartoma 348 11 Osteogenic tumours 259 21 Congenital and inherited syndromes 349 Osteoid osteoma 260 Familial adenomatous polyposis 352 Osteoblastoma 262 Beckwith-Wiedemann syndrome 354 Conventional osteosarcoma 264 Enchondromatosis: Ollier disease Telangiectatic osteosarcoma 271 and Maffucci syndrome 356 Small cell osteosarcoma 273 McCune-Albright syndrome 357 Low grade central osteosarcoma 275 Multiple osteochondromas 360 Secondary osteosarcoma 277 Retinoblastoma syndrome 363 Parosteal osteosarcoma 279 Rothmund-Thomson syndrome 365 Periosteal osteosarcoma 282 Werner syndrome 366 High grade surface osteosarcoma 284 Contributors 369 12 Fibrogenic tumours 287 Source of charts and photographs 374 Desmoplastic fibroma of bone 288 References 376 Fibrosarcoma of bone 289 Subject index 420 bb5_2.qxd 13.9.2006 9:35 Page 9 WHO Classification of Soft Tissue Tumours This new WHO classification of soft tissue tumours, in line with other volumes in this new series, incorporates detailed clinical, histological and genetic data. The explosion of cytogenetic and molecular genetic information in this field over the past 10-15 years has had significant impact on soft tissue tumour classifi- cation and also on our understanding of their biology. The major changes which are reflected