actin 14 adamantinoma 202, 290 292, 297 adenocarcinoma 136 adipocytes in hibernoma 149, 150 in lipoblastoma 148 in lipoma 141, 142, 145 in liposarcoma 152 in myelolipoma 151 adrenal gland tumors see myelolipoma AFH (angiomatoid fibrous histiocytoma) 11, 17, 101 103, 109, 292 294, 297 298 aggressive angiomyxoma 11, 118 119, 134, 135 AIDS 67 see also Kaposi s sarcoma ALK gene 35 ALK-1 protein (anaplastic lymphoma kinase-1) 16 alveolar rhabdomyosarcoma 17, 19, 256 258, 268, 297 alveolar soft part sarcoma (ASPS) 11, 17, 285 288, 295, 297 amyloidoma 239 anaplastic large cell lymphoma 16, 266 ancient schwannoma 69, 70 anesthesia, local, for biopsy 4 aneurysmal bone cyst 105, 307 311 see also telangiectatic osteosarcoma angiogenesis 165 angiolipoma 141, 142 angiomatoid fibrous histiocytoma (AFH) 11, 17, 101 103, 109, 292 294, 297 298 angiomyofibroblastoma 119 120, 134, 135 angiomyxoma, aggressive 11, 118 119, 134, 135 angiosarcoma 11, 174 177, 178 180, 180 182, 296 Antoni A/Antoni B areas in schwannomas 70 arthritis 94 aspiration, as used in FNA biopsy 3, 4 ASPS (alveolar soft part sarcoma) 11, 17, 285 288, 295, 297 ATF1 gene ATF1/EWS 285, 301 ATF1/FUS 294 atypical lipomatous neoplasms see liposarcoma, well differentiated B cell lymphoma see lymphoma (primary lymphoma of bone) Baker s cyst (synovial cyst) 303 304, 310 biopsy deciding which technique to use 1 3 procedures and equipment 3 5 what happens afterwards 5 7 bone cysts aneurysmal 105, 307 311 simple 306 307, 310, 311 see also intraosseous epidermoid cyst; intraosseous ganglion; telangiectatic osteosarcoma bone-forming (osseous) lesions 198 differential diagnosis 135, 198, 213 218, 241 fibrous dysplasia 200 202, 214 fracture callous 214, 217 218 myositis ossificans 198 200, 213, 214 ossifying fibromyxoid tumor 120 121, 135, 215 osteoblastoma 203 204, 214 215 osteofibrous dysplasia (ossifying fibroma) 202, 214 osteoid osteoma 204 205 osteosarcoma see osteosarcoma botryoid embryonal rhabdomyosarcoma 255 256, 258 brown fat tumor (hibernoma) 11, 149 150, 160 161 cambium layer (embryonal rhabdomyosarcoma) 256 capillary hemangioma 167 Carney s triad 226 cartilaginous tumors 220 chondroblastoma 37, 108, 109, 111, 229 232, 241 242 chondroma 220 223, 240, 241, 242 243 extraskeletal 225 226 chondromyxoid fibroma 226 229, 240 241 chondrosarcoma see chondrosarcoma differential diagnosis 37, 108, 109, 111, 215 216, 223, 240 244 cavernous hemangioma 167 CD31 protein 14 CD34 protein 14, 146 CD68 protein 99, 103 CD99 protein 16 CD117 protein 16 17, 83 CDH11 gene 310 cellular schwannoma 69, 70 71 centroblastic lymphoma 266 chenille bodies 48 chondroblastic osteosarcoma 207, 208 209, 211 212, 215, 242 chondroblastoma 229 232 differential diagnosis 37, 108, 109, 111, 241 242 chondroblasts 230, 231, 232 chondrocytes in chondroma 222 in chondrosarcoma 232, 234, 235, 236 in osteochondroma 215, 225 chondroid in chondroblastic osteosarcoma 211 in chondroma 221 in chondrosarcoma 235 compared with osteoid 207, 242 chondroma 220 223, 240, 241, 242 243 extraskeletal 225 226 chondromyxoid fibroma 226 229, 240 241 chondrosarcoma 11, 232 237 clear cell 237 239, 242 dedifferentiated 191 192, 239 differential diagnosis 135, 223, 225, 240, 242 extraskeletal myxoid 11, 17, 127 129, 135, 241, 242 grade I 223, 232, 233 234, 235, 236, 240 grade II 232, 234 235, 236, 240, 241, 243 244 grade III 235, 236, 240, 241 mesenchymal 11, 242, 260 262, 269, 270 periosteal 239 CHOP gene 18, 140 CHOP/FUS 154, 157, 188 CHOP/TLS 127, 156 chordoma 129 132, 135, 136 137, 241, 242 chromosome abnormalities see cytogenetics and molecular diagnostics clear cell chondrosarcoma 237 239, 242 clear cell sarcoma 11, 17, 283 285, 295, 296, 299 301 Codman s triangle 206 conventional giant cell tumor of bone 37, 103 106, 109, 111 112, 241 core biopsy 1, 2 3, 4 5 see also histology cysts 303 bone aneurysmal 105, 307 311 simple 306 307, 310, 311 differential diagnosis 134, 135, 310 312 epidermoid (intraosseous) 305 306, 310 ganglion 114 115, 134, 135, 304, 310, 311 synovial 303 304, 310 telangiectatic osteosarcoma 209, 211, 212, 308, 310, 311 312 cytogenetics and molecular diagnostics 9, 10, 17 adamantinoma aggressive angiomyxoma 119 314
alveolar soft part sarcoma 17, 288 aneurysmal bone cyst 310 angiomatoid fibrous histiocytoma 17, 294 chondroma 223 chondrosarcoma 17, 129, 237, 262 clear cell sarcoma 17, 285, 301 dermatofibrosarcoma protuberans 17, 59 desmoplastic small round cell tumor 17, 260 epithelioid sarcoma, proximal-type 17, 281 Ewing sarcoma/pnet 9, 10, 17 18, 251, 269 fibromatosis 52 fibromyxoid sarcoma, low-grade 17, 122 fibrosarcoma adult-type 61 infantile 17, 32 gastrointestinal stromal tumor 17, 19 20, 83 giant cell fibroblastoma 17, 30 giant cell tumor of bone 106 giant cell tumor of tendon sheath 97, 98 inflammatory myofibroblastic tumor 17, 35 leiomyoma 64 leiomyosarcoma, pleomorphic 194 lipoblastoma 149 lipoma 17, 143, 146, 147 liposarcoma myxoid/round cell 17, 18, 127, 154, 156 well differentiated/dedifferentiated 17, 18, 154, 162, 191 malignant peripheral nerve sheath tumor 76 myxoma 117 nodular fasciitis 44 osteochondroma 225 plexiform fibrohistiocytic tumor 99 rhabdoid tumor 17, 283 rhabdomyosarcoma 17, 19, 258 alveolar 17, 19, 258 synovial sarcoma 17, 18 19, 80 cytokeratins 13, 292 cytology adamantinoma 290 291 aggressive angiomyxoma 118 alveolar soft part sarcoma 286, 297 angiomatoid fibrous histiocytoma 101 102, 293, 297 298 angiomyofibroblastoma 119 angiosarcoma 174 176, 178 180, 180 182, 296 bone cyst aneurysmal 308 309, 310 simple 307, 310 chondroblastoma 37, 112, 229 231, 241 242 chondroma 221 222, 226, 242 243 chondromyxoid fibroma 227 chondrosarcoma 232 236, 243 244 clear cell 237 238 dedifferentiated 192 extraskeletal myxoid 127 128 mesenchymal 261 262 chordoma 130, 136 137 clear cell sarcoma 283 284, 300 dermatofibrosarcoma protuberans 57 58 desmoplastic small round cell tumor 259 elastofibroma dorsi 48 epidermoid cyst (intraosseous) 305 epithelioid sarcoma 278, 295 296 proximal-type 280, 296, 298 Ewing sarcoma 248 249, 271 fibromatosis 50 51 infantile 27 28 fibromatosis colli 23 24, 35 37, 38 fibromyxoid sarcoma, low-grade 76, 121 fibrosarcoma adult-type 60 infantile 31 fibrous dysplasia 201 fibrous hamartoma of infancy 26, 37 fibrous histiocytoma (dermatofibroma) 55 56 foreign body granuloma 92 93, 108 fracture callous 217 218 ganglion cyst 114, 304 gastrointestinal stromal tumor 11, 19 20, 81 giant cell fibroblastoma 29, 37, 99 100, 135 giant cell tumor of bone 103 104, 111 112 giant cell tumor of tendon sheath 95 96, 97 98, 108 glomus tumor/variants 169 grading of sarcomas 6 7 granular cell tumor 160, 275, 294 hemangioendothelioma 172 173 hemangioma 165 166, 168, 178 hibernoma 149, 160 161 inflammatory myofibroblastic tumor 34, 37 Kaposi s sarcoma 171, 178, 180 Langerhans cell histiocytosis 288 289, 297 leiomyoma 62 leiomyosarcoma 64 65, 87 pleomorphic 192 193 lipoblastoma 148 lipoma 140 142 intramuscular 143 144 pleomorphic 147 spindle cell 145 liposarcoma dedifferentiated 159, 191 myxoid/round cell 125 126, 134, 137, 154 155, 162 pleomorphic 157 158, 188 189, 194 196 well differentiated 152 153, 161 lymphoma of bone 265, 271 malignant fibrous histiocytoma/plus 106 107, 109, 183 185 malignant peripheral nerve sheath tumor 74 75 melanoma 180, 300 myelolipoma 150 myeloma 267 132 133 myositis ossificans 198 199 myxofibrosarcoma 85, 123 124 myxoma 115 116, 117 neurofibroma 72 nodular fasciitis 37, 41 43, 85 non-ossifying fibroma 32 33, 37, 38 39 olfactory neuroblastoma 263, 269 ossifying fibromyxoid tumor 120 osteoblastoma 203, 214 osteochondroma 215, 224 225 osteoid osteoma 204 osteosarcoma 206 211, 216 217 chondroblastic 207, 208 209, 242 giant-cell rich 110 111, 210 telangiectatic 209, 211, 310, 312 plexiform fibrohistiocytic tumor 98 99 proliferative fasciitis 46 47 proliferative myositis 44 45 rhabdoid tumor (extra-renal) 282, 296 rhabdomyosarcoma 36, 252, 268, 272 alveolar 256 257, 297 embryonal 252 253 pleomorphic 186 187, 196 rheumatoid nodule 94 schwannoma 68 69, 85 solitary fibrous tumor 52 53 synovial cyst 303 synovial sarcoma 77 78, 86, 277, 294, 299 dedifferentiated tumors see chondrosarcoma, dedifferentiated; liposarcoma, dedifferentiated dermatofibroma (fibrous histiocytoma) 11, 55 57 dermatofibrosarcoma protuberans (DFSP) 11, 17, 57 59 see also giant cell fibroblastoma desmin 14 desmoid tumor see fibromatosis desmoplastic small round cell tumor (DSRCT) 17, 258 260, 269, 270 elastofibroma dorsi 48 49, 84, 85 elderly patients 48, 106, 118, 123, 190 embryonal rhabdomyosarcoma 251, 252 256, 258, 268 encasement sign (chordoma) 222, 223 enchondroma 220 223, 240, 241, 242 243 endothelial differentiation markers 14 15, 146 eosinophilic granuloma 288 epidermoid cyst, intraosseous 305 306, 310 epithelioid gastrointestinal stromal tumor 81, 82 83 epithelioid hemangioendothelioma 172 174, 178, 179, 181 epithelioid hemangioma 166, 167 epithelioid leiomyoma 62, 63 epithelioid leiomyosarcoma 65, 67 epithelioid malignant peripheral nerve sheath tumor 74, 76, 86 epithelioid osteosarcoma 207 epithelioid sarcoma 11, 277 280, 295 296 proximal-type 17, 280 281, 295, 296, 298 epithelioid schwannoma 71 epithelioid tumors 275, 294 301 see also individual types Epstein-Barr virus 67 equipment for core biopsy 4 5 for FNA 3, 4 ETV6/NTRK3 gene fusion 32 Ewing sarcoma 247 251, 268 269, 271 272 adamantinoma-like 290, 292 cytogenetics 9, 10, 17 18, 251, 269, 292 immunohistochemistry 11, 16, 251 EWS gene 9, 17, 251, 269 EWS/ATF1 285, 301 EWS/FLI-1 16, 17 18 EWS/NOR1 129 EWS/WT1 260 EXT1 gene 225 315
familial adenomatous polyposis 50 fasciitis see nodular fasciitis; proliferative fasciitis fat necrosis 142, 160, 161 FHI (fibrous hamartoma of infancy) 25 27, 36, 37 fibroblastoma see giant cell fibroblastoma fibrohistiocytic markers 15, 99, 103 fibrohistiocytic tumor 98 99 fibromatosis 11, 49 52, 84, 85 abdominal 49 50 extra-abdominal 49 infantile 27 29 mesenteric (Gardner s syndrome) 50, 52 fibromyxoid sarcoma, low-grade 12, 17, 76, 85, 121 122, 135 fibromyxoid tumor see ossifying fibromyxoid tumor fibrosarcoma 11 adult-type 59 61 infantile 17, 30 32, 36 fibrous cortical defect (non-ossifying fibroma) 32 34, 36, 37, 38 39 fibrous dysplasia 200 202, 214 fibrous hamartoma of infancy (FHI) 25 27, 36, 37 fibrous histiocytoma (dermatofibroma, sclerosing hemangioma) 11, 55 57 see also angiomatoid fibrous histiocytoma; malignant fibrous histiocytoma fine-needle aspiration (FNA) 1, 2 4 see also cytology FLI-1 protein 16 FLI-1/EWS gene fusion 16, 17 18 floret-like giant cells (pleomorphic lipoma) 147 fluorescence in situ hybridization (FISH) 17, 247 Ewing sarcoma 9, 18, 251 lipoma 143 liposarcoma 18, 127, 154, 156 rhabdomyosarcoma, alveolar 19, 258 synovial sarcoma 19 FNCLCC (Nationale des Centres de Lutte Contre le Cancer) grading system 6 foreign body granuloma 92 93, 108, 109 FOXO1A gene 19 fracture callous 214, 217 218 FUS gene FUS/ATF-1 294 FUS/CHOP 154, 157, 188 ganglion cyst 114 115, 134, 135, 304, 310, 311 ganglion-like cells 46 47 Gardner s syndrome (mesenteric fibromatosis) 50, 52 gastrointestinal stromal tumor (GIST) 11, 17, 19 20, 80 83, 84 genetics see cytogenetics and molecular diagnostics giant cell tumors 92 angiomatoid fibrous histiocytoma 11, 17, 101 103, 109, 292 294, 297 298 of bone 37, 103 106, 109, 111 112, 241 differential diagnosis 36, 37, 108 112, 135, 241, 297 298 foreign body granuloma 92 93, 108, 109 giant cell fibroblastoma 11, 17, 29 30, 36, 37, 99 101, 109, 135 malignant fibrous histiocytoma/plus 106 108, 109, 183 186 osteosarcoma 109 111, 210 plexiform fibrohistiocytic tumor 98 99 rheumatoid nodule 93 94 of tendon sheath 94 95, 109 diffuse 97 98, 108 localized 95 97, 108 GIST (gastrointestinal stromal tumor) 11, 17, 19 20, 80 83, 84 Gleevec (imatinib) 19 20 glomus tumor/glomangioma/glomangiomyoma 169 170, 178, 179 grading, of sarcomas 5 7 granular cell tumor 11, 160, 275 277, 294, 295 granulation tissue 178, 179 granuloma eosinophilic 288 foreign body type 92 93, 108, 109 growth factors 165 H-caldesmon 194 Hand-Schuller-Christian disease 288 hemangioendothelioma, epithelioid 172 174, 178, 179, 181 hemangioma 165 168, 178, 179 of bone 168 169 hemangiopericytoma 52, 54, 169 see also solitary fibrous tumor hemosiderin in aneurysmal bone cyst 309 in angiosarcoma 180 in fibrous histiocytoma 56, 102 in giant cell tumor of bone 106 in giant cell tumor of tendon sheath 95, 96, 97, 98, 108 in Kaposi s sarcoma 172 herpesviruses 170 hibernoma 11, 149 150, 160 161 high resolution melting amplicon analysis 17, 20, 83 histiocytes and histiocyte-like cells in angiomatoid fibrous histiocytoma 102, 293 294 in dermatofibrosarcoma protuberans 58 epithelioid 92, 93, 94 in fibrous histiocytoma 56, 184, 185 foamy (xanthoma cells) 34, 56, 96, 109, 161 in synovial cyst 303 see also giant cell tumors histiocytoma see angiomatoid fibrous histiocytoma; fibrous histiocytoma histology adamantinoma 292 aggressive angiomyxoma 118 alveolar soft part sarcoma 287 angiomatoid fibrous histiocytoma 102 103, 293 294 angiomyofibroblastoma 119 angiosarcoma 176 177, 181 bone cyst aneurysmal 309 310, 311 simple 307 chondroblastoma 231 232 chondroma 222 223, 226 chondromyxoid fibroma 228 229 chondrosarcoma 223, 236 237 clear cell 238 239 dedifferentiated 192 extraskeletal myxoid 128 mesenchymal 262 chordoma 131 clear cell sarcoma 284 285 dermatofibrosarcoma protuberans 58 59 desmoplastic small round cell tumor 260 elastofibroma dorsi 48 epidermoid cyst (intraosseous) 306 epithelioid sarcoma 279 280 proximal-type 281 Ewing sarcoma 250 fibromatosis 51 52 infantile 28 fibromatosis colli 24 25 fibromyxoid sarcoma, low-grade 122 fibrosarcoma adult-type 61 infantile 31 fibrous dysplasia 201 fibrous hamartoma of infancy 26 27 fibrous histiocytoma (dermatofibroma) 56 57 foreign body granuloma 93 ganglion cyst 115, 304 gastrointestinal stromal tumor 82 83 giant cell fibroblastoma 30, 100 101 giant cell tumor of bone 105 106 giant cell tumor of tendon sheath 96, 98 glomus tumor/variants 169 170 grading of sarcomas 5 6 granular cell tumor 276 277 hemangioendothelioma 173 174 hemangioma 167, 168 hibernoma 150 inflammatory myofibroblastic tumor 35 Kaposi s sarcoma 171 172 Langerhans cell histiocytosis 289 290 leiomyoma 63 64 leiomyosarcoma 66 67, 87 pleomorphic 193 194 lipoblastoma 148 149 lipoma 142 143, 144 pleomorphic 147 spindle cell 145 146 liposarcoma dedifferentiated 159, 191 myxoid/round cell 126 127, 149, 156, 162 pleomorphic 158, 189 190 well differentiated 153, 161 lymphoma of bone 266 malignant fibrous histiocytoma/plus 107, 185 186 malignant peripheral nerve sheath tumor 75 76 myelolipoma 151 myeloma 267 268 133 134 myositis ossificans 199 200 myxofibrosarcoma 124 myxoma 116, 117 neurofibroma 72 73 316
nodular fasciitis 43 44 non-ossifying fibroma 33 34 olfactory neuroblastoma 263 264 ossifying fibromyxoid tumor 120 121 osteoblastoma 203 204 osteochondroma 225 osteoid osteoma 205 osteosarcoma 211 213 plexiform fibrohistiocytic tumor 99 proliferative fasciitis 47 proliferative myositis 45 46 rhabdoid tumor (extra-renal) 283 rhabdomyosarcoma alveolar 257 embryonal 253 256 pleomorphic 187 188 rheumatoid nodule 94 schwannoma 70 71 solitary fibrous tumor 54 55 synovial cyst 303 synovial sarcoma 79 80, 86 HMB45 antibody 15 HMGA2 gene 119 imaging see radiography imatinib mesylate 19 20 immunoblastic lymphoma 266 immunohistochemistry 9 17 adamantinoma 292 aggressive angiomyxoma 11, 118 alveolar soft part sarcoma 11, 287 angiomatoid fibrous histiocytoma 11, 103, 294 angiomyofibroblastoma 119 angiosarcoma 11, 177 bone cyst, aneurysmal 310 chondroblastoma 232 chondroma 223 chondromyxoid fibroma 229 chondrosarcoma 11, 237 clear cell 239 dedifferentiated 192 extraskeletal myxoid 11, 129 mesenchymal 11, 262 chordoma 131, 137 clear cell sarcoma 11, 285 dermatofibrosarcoma protuberans 11, 59 desmoplastic small round cell tumor 260 epithelioid sarcoma 11, 280, 281, 296 Ewing sarcoma/pnet 11, 16, 251 fibromatosis 11, 52 fibromatosis colli 25 fibromyxoid sarcoma, low-grade 12, 122 fibrosarcoma 11, 32, 61 fibrous hamartoma of infancy 27 fibrous histiocytoma (dermatofibroma) 11, 57 gastrointestinal stromal tumor 11, 83 giant cell fibroblastoma 11, 30, 101 giant cell tumor of bone 106 giant cell tumor of tendon sheath 97, 98 glomus tumor/variants 170 granular cell tumor 11, 277 hemangioendothelioma 174 hemangioma 167, 169 hibernoma 11, 150 inflammatory myofibroblastic tumor 35 Kaposi s sarcoma 172 Langerhans cell histiocytosis 290 leiomyoma 64 leiomyosarcoma 11, 67 pleomorphic 194 lipoma pleomorphic 12, 147 spindle cell 12, 146 liposarcoma 11, 158, 159, 190 lymphoma of bone 12, 266 malignant fibrous histiocytoma/plus 107, 186 malignant peripheral nerve sheath tumor 12, 76, 86 myeloma 268 134 myxoma 12, 117 neurofibroma 73 nodular fasciitis 44 non-ossifying fibroma 34 olfactory neuroblastoma 264 ossifying fibromyxoid tumor 121 osteosarcoma 213 pleomorphic sarcoma 194 plexiform fibrohistiocytic tumor 99 proliferative fasciitis 47 proliferative myositis 46 rhabdoid tumor (extra-renal) 11, 283, 296 rhabdomyosarcoma 12, 188, 258 schwannoma 12, 71 solitary fibrous tumor 12, 55 synovial sarcoma 12, 80, 87 incisional biopsy 2 infants fibrous hamartoma of infancy 25 27, 36, 37 infantile fibromatosis 27 29 infantile fibrosarcoma 17, 30 32, 36 infections in Kaposi s sarcoma 170 testing for 93, 94 inflammatory liposarcoma 152, 153 inflammatory myofibroblastic tumor (IMT) 17, 34 35, 36, 37 inflammatory pseudo tumor 34 intermediate filament proteins 13 14, 292 intraosseous epidermoid cyst 305 306, 310 intraosseous ganglion 306 intraosseous hemangioma 168 169 Kaposi s sarcoma 170 172, 178, 179, 180 karyotype 9 keratin (cytokeratins) 13, 292 Kimura s disease 166 KIT gene 19 20, 83 see also CD117 Langerhans cell histiocytosis 288 290, 295, 297 leiomyoma 61 64, 86 leiomyosarcoma 11, 64 67 differential diagnosis 64, 84, 86, 87 88, 195 pleomorphic 192 194, 195 Letterer-Siwe disease 288 lipoblastoma 148 149, 160, 161 lipoblasts in myxoid liposarcoma 125, 126, 137, 154 in pleomorphic liposarcoma 157, 158, 161, 189 in well differentiated liposarcoma 153 lipoma 140 143 angiolipoma 141, 142 differential diagnosis 140, 142, 143, 160 intramuscular 143 144 myelolipoma 150 151, 160 myolipoma 141, 142, 143 pleomorphic 12, 146 148, 160 spindle cell 12, 17, 144 146, 160 see also hibernoma lipoma-like liposarcoma 152, 153 lipomatous tumors, differential diagnosis 140, 160 163 liposarcoma 11 dedifferentiated 18, 158 159, 190 191 differential diagnosis 134, 137 138, 142, 158, 160, 161 163 myxoid 124 127, 154, 155 cytogenetics 17, 18, 127, 154, 156 differential diagnosis 134, 135, 137 138, 142, 149, 160, 161, 162 163 pleomorphic 157 158, 160, 161, 188 190, 194 196 round cell 154, 155 156, 160 well differentiated 17, 18, 151 154, 160, 161 162 lymphoma (primary lymphoma of bone) 12, 264 266, 269, 271 Maffucci syndrome 223 magnetic resonance imaging (MRI) aneurysmal bone cyst 308 desmoid tumor 50 ganglion cyst 114 giant cell tumor of tendon sheath 95, 97 hemangioma 165 lipoma 140, 143 malignant peripheral nerve sheath tumor 74 neurofibroma 72 schwannoma 68 malignant fibrous histiocytoma (MFH) (pleomorphic undifferentiated sarcoma, PLUS) 106 108, 109, 183 186 see also angiomatoid fibrous histiocytoma; fibrous histiocytoma malignant peripheral nerve sheath tumor (malignant schwannoma) 12, 73 76, 84, 86, 195 McCune-Albright syndrome 200 MDM2 gene 18, 143, 147, 154, 162, 191 melan-a (MART-1) protein 15, 190 melanoma 15, 195, 299 301 compared with angiosarcoma 180 compared with ASPS 297 compared with clear cell chondrosarcoma 242 compared with clear cell sarcoma 296 compared with pleomorphic liposarcoma 190, 194 melanoma of soft parts (clear cell sarcoma) 11, 17, 283 285, 295, 296, 299 301 melanosomal markers 15 16 317
mesenchymal chondrosarcoma 11, 242, 260 262, 269, 270 mesothelioma 177 MFH see malignant fibrous histiocytoma molecular diagnostics see cytogenetics and molecular diagnostics muscle differentiation markers 13 14 myelolipoma 150 151, 160 myeloma 266 268, 269, 271, 296 MyoD1 protein 14 myofibroblastic tumor, inflammatory 17, 34 35, 36, 37 myogenin 14 132 134 myolipoma 141, 142, 143 myositis, proliferative 44 46 myositis ossificans 198 200, 213, 214 myxoid tumors 114 aggressive angiomyxoma 11, 118 119, 134, 135 angiomyofibroblastoma 119 120, 134, 135 chondrosarcoma 11, 17, 127 129, 135, 241, 242 chordoma 129 132, 135, 136 137, 241, 242 differential diagnosis 134 138, 215, 242 chondrosarcoma/chordoma 241, 242 liposarcoma 142, 149, 160, 161, 162 163 fibromyxoid sarcoma, low-grade 12, 17, 76, 85, 121 122, 135 ganglion cyst 114 115, 134, 135, 304, 310, 311 liposarcoma 17, 18, 124 127, 154 156 differential diagnosis 134, 135, 137 138, 142, 149, 160, 161, 162 163 132 134 myxofibrosarcoma 12, 85, 123 124, 134, 135 myxoma 12, 134, 135 intramuscular 115 117 juxta-articular 117 ossifying fibromyxoid tumor 120 121, 135, 215 NCC (National Comprehensive Cancer) Network guidelines 5 NCI (National Cancer Institute) grading system 5 6 needles used in core biopsy 4 5 used in FNA 3, 4 neurilemoma see schwannoma neuroblastoma 269 olfactory 262 264, 269, 270 neurofibroma 71 73 neurofibromatosis type I 71 72, 74 neurofibromatosis type II 68, 72 neurofibrosarcoma see malignant peripheral nerve sheath tumor nodular fasciitis 37, 41 44, 84, 85 nodular tenosynovitis see tendon sheath; giant cell tumors non-ossifying fibroma (fibrous cortical defect) 32 34, 36, 37, 38 39 olfactory neuroblastoma 262 264, 269, 270 Ollier s disease 223 osseous tumors 198 differential diagnosis 135, 198, 213 218, 241 fibromyxoid tumor 120 121, 135, 215 fibrous dysplasia 200 202, 214 fracture callous 214, 217 218 myositis ossificans 198 200, 213, 214 osteoblastoma 203 204, 214 215 osteofibrous dysplasia (ossifying fibroma) 202, 214 osteoid osteoma 204 205 osteosarcoma see osteosarcoma osteoblastic differentiation markers 15 osteoblastoma 203 204, 214 215 osteoblasts in myositis ossificans 199 in osteoblastoma 203 in osteoid osteoma 205 in osteosarcoma 110, 206 207, 210, 211, 217 osteocalcin 15 osteoclast-like giant cells in aneurysmal bone cyst 309 in chondroblastoma 231, 232 in giant cell tumor of bone 103, 104, 105, 111 in giant cell tumor of tendon sheath 95, 98 in malignant fibrous histiocytoma/plus 106, 107, 184 in myositis ossificans 199 in non-ossifying fibroma 34 in osteosarcoma 110, 111, 207, 210 osteofibrous dysplasia (ossifying fibroma) 202, 214 osteoid in aneurysmal bone cyst 309 in clear cell chondrosarcoma 238 compared with chondroid 206, 242 in osteosarcoma 212, 217, 242 osteoid osteoma 204 205 osteonectin 15, 239 osteosarcoma 205 213 differential diagnosis 36, 198, 214, 215, 216 217, 242 giant-cell rich 109, 111 telangiectatic 308, 310, 311 312 subtypes chondroblastic 207, 208 209, 211 212, 215, 242 epithelioid 207 giant-cell rich 109 111, 210 high-grade intramedullary 206 207, 210, 211, 214 low-grade central/intramedullary 210, 211, 213, 214 parosteal 210, 211, 212 periosteal 208 209, 211, 215 pleomorphic 207 small cell 207, 209, 212 telangiectatic 209, 211, 212, 308, 310, 311 312 parosteal osteosarcoma 210, 211, 212 PAX3/PAX7-FOXO1A gene fusion 19 PDGFRA gene 83 PECAM-1 see CD31 protein pediatric tumors 23, 35 39 bone cysts 306 chondroma 220 fibrous hamartoma of infancy 25 27, 36, 37 giant cell fibroblastoma 11, 17, 29 30, 99 101 differential diagnosis 36, 37, 109, 135 hemangioma 165, 178 infantile fibromatosis 27 29 infantile fibrosarcoma 17, 30 32, 36 inflammatory myofibroblastic tumor 17, 34 35, 36, 37 Langerhans cell histiocytosis 288 lipoblastoma 148 149, 160, 161 non-ossifying fibroma 32 34, 36, 37, 38 39 osteoblastoma 203 osteochondroma 215 216, 223 225, 241 osteosarcoma 205 retinoblastoma 64, 67 rhabdomyosarcoma (embryonal form) 251, 252 256, 258, 268 periosteal chondroma (parosteal chondroma) 220 periosteal chondrosarcoma 239 periosteal osteosarcoma 208 209, 211, 215 peripheral nerve sheath tumor, malignant 12, 73 76, 84, 86, 195 permeation pattern (chondrosarcoma) 223 physaliferous cells (chordoma) 130, 131, 136, 242 pigmented villonodular synovitis (diffuse giant cell tumor of tendon sheath) 97 98, 108 pilar leiomyoma 61, 62, 63, 64 plasmacytoma (myeloma) 266 268, 269, 271, 296 pleomorphic carcinoma 183, 194, 195 pleomorphic leiomyosarcoma 192 194, 195 pleomorphic lipoma 12, 146 148, 160 pleomorphic liposarcoma 157 158, 160, 161, 188 190, 194 196 pleomorphic osteosarcoma 207 pleomorphic rhabdomyosarcoma 186 188, 195, 196 pleomorphic sarcomas 183, 194 196 see also individual tumor types pleomorphic undifferentiated sarcoma (PLUS) (malignant fibrous histiocytoma, MFH) 106 108, 109, 183 186 plexiform fibrohistiocytic tumor 98 99 primitive neuroectodermal tumor (PNET) 247 248, 249, 250 251, 268 269 cytogenetics 17 18, 251 see also Ewing sarcoma prognosis 5, 13 gastrointestinal stromal tumor 82 leiomyosarcoma 64 proliferative fasciitis 46 47, 85 proliferative myositis 44 46 radiography 213 adamantinoma 290, 297 bone cyst aneurysmal 308, 310 simple 307 chondroblastoma 229 chondroma 220 221, 226, 242 chondromyxoid fibroma 226 chondrosarcoma 192, 232, 237, 243, 261 chordoma 129, 136 Ewing sarcoma 248 fibromatosis (desmoid tumor) 50 fibrous dysplasia 200 201 318
fracture callous 217 ganglion cyst 114 giant cell tumor of bone 103, 111 giant cell tumor of tendon sheath 95, 97 hemangioma 165, 168 Langerhans cell histiocytosis 288 lipoma 140, 143 liposarcoma 124, 152, 154, 159, 191 lymphoma of bone 264 malignant peripheral nerve sheath tumor 74 myeloma 266 myositis ossificans 198 neurofibroma 72 non-ossifying fibroma 32, 38 osteoblastoma 203, 214 osteochondroma 215, 224 osteoid osteoma 204 osteosarcoma 198, 205, 206, 216 giant-cell rich 109 telangiectatic 311 schwannoma 68 synovial sarcoma 77, 277 radiotherapy 7 Reed-Sternberg-like cells 133, 134 renal cell carcinoma 160, 242, 294, 297 retinoblastoma 64, 67 rhabdoid tumor 17, 295 extra-renal 11, 278, 281 283, 296 rhabdomyoma 12, 294 rhabdomyosarcoma 12, 251 252 alveolar 17, 19, 256 258, 268, 297 classification 251 252 differential diagnosis 36, 196, 268, 269, 272 273, 297 embryonal 252 256, 258, 268 pleomorphic 186 188, 195, 196 rheumatoid nodule 93 94 rosettes in Ewing sarcoma/pnet 249, 250, 269, 271 in fibromyxoid sarcoma 122 in olfactory neuroblastoma 264, 269 round cell liposarcoma 154 156, 160 S-100 protein 15 16 sample preparation 4 sarcoma botryoides 255 256, 258 sarcomas grading 5 7 selection of treatment 7 see also individual tumor types schwannoma (neurilemoma) 12, 67 71, 84, 85 86 sclerosing hemangioma (fibrous histiocytoma) 11, 55 57 sclerosing liposarcoma 152, 153 small round cell neoplasms 247 desmoplastic 17, 258 260, 269, 270 differential diagnosis 36, 268 273, 296, 297 Ewing sarcoma/pnet see Ewing sarcoma lymphoma of bone 12, 264 266, 269, 271 mesenchymal chondrosarcoma 11, 242, 260 262, 269, 270 myeloma 266 268, 269, 271, 296 olfactory neuroblastoma 262 264, 269, 270 osteosarcoma 207, 209, 212 rhabdomyosarcoma see rhabdomyosarcoma solitary fibrous tumor 12, 52 55, 84, 85 somatic leiomyoma 61, 63 specimen preparation 4 spindle cell tumors 23, 41 dermatofibrosarcoma protuberans 11, 17, 57 59 differential diagnosis 35 39, 83 88, 109, 135, 160, 195, 295 elastofibroma dorsi 48 49, 84, 85 fibromatosis 11, 49 52, 84, 85 infantile 27 29 fibromyxoid sarcoma, low-grade 12, 17, 76, 85, 121 122, 135 fibrosarcoma 11 adult-type 59 61 infantile 17, 30 32, 36 fibrous hamartoma of infancy 25 27, 36, 37 fibrous histiocytoma 11, 55 57 gastrointestinal stromal tumor 11, 17, 19 20, 80 83, 84 giant cell fibroblastoma 11, 17, 29 30, 99 101 differential diagnosis 36, 37, 109, 135 hemangioma 167 inflammatory myofibroblastic tumor 17, 34 35, 36, 37 leiomyoma 61 64, 86 leiomyosarcoma 11, 64 67, 84, 86, 87 88 see also pleomorphic leiomyosarcoma lipoma 12, 17, 144 146, 160 see also pleomorphic lipoma malignant peripheral nerve sheath tumor 12, 73 76, 84, 86, 195 neurofibroma 71 73 nodular fasciitis 37, 41 44, 84, 85 non-ossifying fibroma 32 34, 36, 37, 38 39 proliferative fasciitis 46 47, 85 proliferative myositis 44 46 schwannoma 67 71, 84, 85 86 solitary fibrous tumor 12, 52 55, 84, 85 synovial sarcoma 12, 17, 18 19, 76 80, 277 differential diagnosis 84, 86 87, 294 295, 298 299 spine hemangioma 168 osteoblastoma 203 staging of tumors 1 storiform pleomorphic malignant fibrous histiocytoma 108, 109, 185 synovial cyst 303 304, 310 synovial sarcoma 12, 17, 18 19, 76 80, 277 differential diagnosis 84, 86 87, 294 295, 298 299 SYT-SSX gene fusion 18 19, 80 TEL gene fusion 32 telangiectatic osteosarcoma 209, 211, 212, 310 differential diagnosis 308, 310, 311 312 tendon sheath, giant cell tumors 94 95, 109 diffuse 97 98, 108 localized 95 97, 108 thrombomodulin 15 tigroid pattern (rhabdomyosarcoma) 253 TLS-CHOP gene fusion 127, 156 torticollis, infantile (fibromatosis colli) 23 25, 35 37, 38 TPM3-ALK and TPM4-ALK gene fusions 35 translocations 17 aggressive angiomyxoma 119 alveolar soft part sarcoma 288 aneurysmal bone cyst 310 chondrosarcoma 129, 262 clear cell sarcoma 285, 301 dermatofibrosarcoma protuberans 59 Ewing sarcoma/pnet 10, 17 18, 251, 292 fibromyxoid sarcoma, low-grade 122 giant cell fibroblastoma 30 giant cell tumor of tendon sheath 97 infantile fibrosarcoma 32 lipoma 143 myxoid liposarcoma 18, 127, 154 rhabdomyosarcoma, alveolar 19, 258 synovial sarcoma 18 19, 80 treatment, selection of 1, 5, 7 Triton tumor 76 Ulex europeus 1 agglutinin 15 USP6 gene 310 vascular tumors 165 angiosarcoma 11, 174 177, 178 180, 180 182, 296 differential diagnosis 177 182, 296 glomus tumor/variants 169 170, 178, 179 hemangioendothelioma 172 174, 178, 179, 181 hemangioma 165 169, 178, 179 Kaposi s sarcoma 170 172, 178, 179, 180 leiomyosarcoma 64, 67 Verhoeff stain (elastin stain) 48, 49 Verocay bodies 69, 70, 82, 85 vertebral hemangioma 168 vertebral osteoblastoma 203 vestibular schwannoma 68 vimentin 13 Wagner-Meissner bodies 73 Wilms tumor 269 WT1 protein 16 WT1/EWS gene fusion 260 X-rays see radiography xanthoma cells (foamy histiocytes) 34, 56, 96, 109, 161 xanthomatous change in giant cell tumor of bone 104, 106 319