Contents
List of abbreviations ix
Foreword x
1 Soft tissue tumours 1
WHO classification of soft tissue tumours 2
TNM staging of tumours of soft tissues 4
TNM staging of gastrointestinal stromal tumours 5
Introduction 6
Adipocytic tumours
Lipoma 13
Lipomatosis 16
Lipomatosis of nerve 18
Lipoblastoma and lipoblastomatosis 20
Angiolipoma 23
Myolipoma of soft tissue 25
Chondroid lipoma 27
Spindle cell lipoma and pleomorphic lipoma 29
Hibernoma 31
Atypical spindle cell / pleomorphic lipomatous tumour 34
Atypical lipomatous tumour / well-differentiated
liposarcoma 36
Dedifferentiated liposarcoma 39
Myxoid liposarcoma 42
Pleomorphic liposarcoma 45
Myxoid pleomorphic liposarcoma 47
Fibroblastic and myofibroblastic tumours
Nodular fasciitis 49
Proliferative fasciitis and proliferative myositis 51
Myositis ossificans and fibro-osseous pseudotumour of
digits 53
Ischaemic fasciitis 55
Elastofibroma 57
Fibrous hamartoma of infancy 59
Fibromatosis colli 61
Juvenile hyaline fibromatosis 63
Inclusion body fibromatosis 65
Fibroma of tendon sheath 67
Desmoplastic fibroblastoma 69
Myofibroblastoma 71
Calcifying aponeurotic fibroma 74
EWSR1-SMAD3–positive fibroblastic tumour (emerging) 76
Angiomyofibroblastoma 78
Cellular angiofibroma 80
Angiofibroma of soft tissue 82
Nuchal-type fibroma 84
Acral fibromyxoma 86
Gardner fibroma 88
Palmar fibromatosis and plantar fibromatosis 90
Desmoid fibromatosis 93
Lipofibromatosis 96
Giant cell fibroblastoma 98
Dermatofibrosarcoma protuberans 100
Solitary fibrous tumour 104
Inflammatory myofibroblastic tumour 109
Low-grade myofibroblastic sarcoma 112
Superficial CD34-positive fibroblastic tumour 114
Myxoinflammatory fibroblastic sarcoma 116
Infantile fibrosarcoma 119
Adult fibrosarcoma 122
Myxofibrosarcoma 124
Low-grade fibromyxoid sarcoma 127
Sclerosing epithelioid fibrosarcoma 130
So-called fibrohistiocytic tumours
Tenosynovial giant cell tumour 133
Deep fibrous histiocytoma 137
Plexiform fibrohistiocytic tumour 139
Giant cell tumour of soft tissue 141
Vascular tumours
Haemangiomas
Synovial haemangioma 143
Intramuscular angioma 145
Arteriovenous malformation/haemangioma 147
Venous haemangioma 149
Anastomosing haemangioma 150
Epithelioid haemangioma 152
Lymphangioma and lymphangiomatosis 154
Tufted angioma and kaposiform haemangioendothelioma 156
Retiform haemangioendothelioma 159
Papillary intralymphatic angioendothelioma 161
Composite haemangioendothelioma 163
Kaposi sarcoma 166
Pseudomyogenic haemangioendothelioma 169
Epithelioid haemangioendothelioma 172
Angiosarcoma 176
Pericytic (perivascular) tumours
Glomus tumour 179
Myopericytoma, including myofibroma 182
Angioleiomyoma 186
Smooth muscle tumours
Leiomyoma 188
EBV-associated smooth muscle tumour 190
Inflammatory leiomyosarcoma 193
Leiomyosarcoma 195
Skeletal muscle tumours
Rhabdomyoma 198
Embryonal rhabdomyosarcoma 201
Alveolar rhabdomyosarcoma 205
Pleomorphic rhabdomyosarcoma 209
Spindle cell / sclerosing rhabdomyosarcoma 211
Ectomesenchymoma 214
Gastrointestinal stromal tumour 216
Chondro-osseous tumours
Soft tissue chondroma 222
Extraskeletal osteosarcoma 224
Peripheral nerve sheath tumours
Schwannoma 226
Neurofibroma 232
Perineurioma 237
Granular cell tumour 240
Dermal nerve sheath myxoma 243
Solitary circumscribed neuroma 245
Ectopic meningioma and meningothelial hamartoma 247
Benign triton tumour / neuromuscular choristoma 249
Hybrid nerve sheath tumour 252
Malignant peripheral nerve sheath tumour 254
Malignant melanotic nerve sheath tumour 258
Tumours of uncertain differentiation
Intramuscular myxoma 261
Juxta-articular myxoma 264
Deep (aggressive) angiomyxoma 266
Atypical fibroxanthoma 268
Angiomatoid fibrous histiocytoma 271
Ossifying fibromyxoid tumour 274
Myoepithelioma, myoepithelial carcinoma, and mixed
tumour 277
Pleomorphic hyalinizing angiectatic tumour of soft parts 280
Haemosiderotic fibrolipomatous tumour 282
Phosphaturic mesenchymal tumour 284
NTRK-rearranged spindle cell neoplasm (emerging) 287
Synovial sarcoma 290
Epithelioid sarcoma 294
Alveolar soft part sarcoma 297
Clear cell sarcoma of soft tissue 300
Extraskeletal myxoid chondrosarcoma 303
Desmoplastic small round cell tumour 306
Extrarenal rhabdoid tumour 309
PEComa 312
Intimal sarcoma 315
Undifferentiated sarcoma 318
2 Undifferentiated small round cell sarcomas of bone and
soft tissue 321
WHO classification of undifferentiated small round cell
sarcomas of bone and soft tissue 322
Ewing sarcoma 323
Round cell sarcoma with EWSR1–non-ETS fusions 326
CIC-rearranged sarcoma 330
Sarcoma with BCOR genetic alterations 333
3 Bone tumours 337
WHO classification of bone tumours 338
TNM staging of tumours of bone 339
Introduction 340
Chondrogenic tumours
Subungual exostosis 345
Bizarre parosteal osteochondromatous proliferation 348
Periosteal chondroma 351
Enchondroma 353
Osteochondroma 356
Chondroblastoma 359
Chondromyxoid fibroma 362
Osteochondromyxoma 365
Synovial chondromatosis 368
Central atypical cartilaginous tumour / chondrosarcoma,
grade 1 370
Secondary peripheral atypical cartilaginous tumour /
chondrosarcoma, grade 1 373
Central chondrosarcoma, grades 2 and 3 375
Secondary peripheral chondrosarcoma, grades 2 and 3 379
Periosteal chondrosarcoma 381
Clear cell chondrosarcoma 383
Mesenchymal chondrosarcoma 385
Dedifferentiated chondrosarcoma 388
Osteogenic tumours
Osteoma 391
Osteoid osteoma 394
Osteoblastoma 397
Low-grade central osteosarcoma 400
Osteosarcoma 403
Parosteal osteosarcoma 410
Periosteal osteosarcoma 414
High-grade surface osteosarcoma 417
Secondary osteosarcoma 419
Fibrogenic tumours
Desmoplastic fibroma of bone 422
Fibrosarcoma of bone 424
Vascular tumours of bone
Haemangioma of bone 426
Epithelioid haemangioma of bone 428
Epithelioid haemangioendothelioma of bone 431
Angiosarcoma of bone 434
Osteoclastic giant cell–rich tumours
Aneurysmal bone cyst 437
Giant cell tumour of bone 440
Non-ossifying fibroma 447
Notochordal tumours
Benign notochordal cell tumour 449
Conventional chordoma 451
Dedifferentiated chordoma 454
Poorly differentiated chordoma 456
Other mesenchymal tumours of bone
Chondromesenchymal hamartoma of chest wall 458
Osteofibrous dysplasia 460
Adamantinoma of long bones 463
Simple bone cyst 467
Fibrocartilaginous mesenchymoma 470
Fibrous dysplasia 472
Lipoma and hibernoma of bone 475
Leiomyosarcoma of bone 478
Undifferentiated pleomorphic sarcoma 480
Bone metastases 483
Haematopoietic neoplasms of bone
Solitary plasmacytoma of bone 486
Primary non-Hodgkin lymphoma of bone 489
Langerhans cell histiocytosis 492
Erdheim–Chester disease 495
Rosai–Dorfman disease 498
4 Genetic tumour syndromes of soft tissue and bone 501
Introduction 502
Enchondromatosis 506
Li–Fraumeni syndrome 510
McCune–Albright syndrome 514
Multiple osteochondromas 517
Neurofibromatosis type 1 520
Rothmund–Thomson syndrome 522
Werner syndrome 525
Contributors 528
Declaration of interests 535
IARC/WHO Committee for ICD-O 536
Sources 537
References 544
Subject index 598
Previous volumes in the series 607