Cambridge University Press 978-0-521-87409-0 - Modern Soft Tissue Pathology: Tumors and Non-Neoplastic Conditions Edited by Markku Miettinen Index More information

Index

abdominal ependymoma, 744 mucinous cystadenocarcinoma, 631 adult fibrosarcoma (AF), 364–365, 1026 abdominal extrauterine smooth muscle ovarian adenocarcinoma, 72, 79 adult granulosa cell tumor, 523–524 tumors, 79 pancreatic adenocarcinoma, 846 clinical features, 523 abdominal inflammatory myofibroblastic pulmonary adenocarcinoma, 51 genetics, 524 tumors, 297–298 renal adenocarcinoma, 67 pathology, 523–524 abdominal leiomyoma, 467, 477 serous cystadenocarcinoma, 631 adult rhabdomyoma, 548–549 abdominal leiomyosarcoma. See urinary bladder/urogenital tract clinical features, 548 gastrointestinal stromal tumor adenocarcinoma, 72, 401 differential diagnosis, 549 (GIST) uterine adenocarcinomas, 72 genetics, 549 abdominal perivascular epithelioid cell tumors adenofibroma, 523 pathology, 548–549 (PEComas), 542 adenoid cystic carcinoma, 1035 aggressive angiomyxoma (AAM), 514–518 abdominal wall desmoids, 244 adenomatoid tumor, 811–813 clinical features, 514–516 acquired elastotic hemangioma, 598 adenomatous polyposis coli (APC) gene, 143 differential diagnosis, 518 acquired tufted angioma, 590 adenosarcoma (mullerian¨ adenosarcoma), 523 genetics, 518 acral arteriovenous tumor, 583 adipocytic lesions (cytology), 1017–1022 pathology, 516 acral myxoinflammatory fibroblastic sarcoma atypical lipomatous tumor/well- aggressive digital papillary adenocarcinoma, (AMIFS), 365–370, 1026 differentiated liposarcoma, 1021 799 clinical features, 365–367 fat necrosis (FN), 1018 AIDS/HIV infection, 401, 423, 636. See also differential diagnosis, 370 hibernoma, 1019–1021 epidemic Kaposi’s sarcoma genetics, 370 lipoblastoma (LB), 1018–1019 alkaptonuric ochronosis, 950–951 pathology, 368 lipoma, 1017–1018 ALK (anaplastic lymphoma kinase) gene acroangiodermatitis (pseudo-Kaposi’s myxoid/round cell liposarcoma,1021–1022 arrangements, 134–136, 138–139 sarcoma), 603 myxolipoma (ML), 1018 alpha-1-antichymotrypsin (AACT), 67 actinic keratosis, 836 spindle cell/pleomorphic lipoma (SC/PL), alpha-1-antitrypsin (AAT), 67 actinomycin D, 1064 1019 alpha-internexin, 59 actinomycosis, 202 adrenal pheochromocytoma, 58, 662, 758–761 alpha smooth muscle actin (SMA), 52–53 actins, 51–52 clinical features, 758 Alport’s syndrome, 467, 469 acute bartonellosis, 602–603 genetic syndrome, 758–760 alveolar rhabdomyosarcoma, 56, 84, 110, 551, acute lymphoblastic leukemias (ALLs), 131 immunohistochemistry, 760–761 559–561, 1037–1038 acute myeloid leukemia (AML), 453, 994–996 malignancy prediction, 760 differential diagnosis, 918, 1037–1038, FOX gene fusion in, 133 non–syndrome-related genetics, 761 1051 FUS-ERG gene fusion in, 129 pathology, 758 genetics, 138–139 WT1 overexpression in, 143 ultrastructure, 761 histopathology, 559–560 acute purulent synovitis (septic arthritis), adrenal tumors. See also adrenal MYCN amplification in, 138–139 939–940 pheochromocytoma PAX3-AFX/PAX3-NCOA1 fusion genes adenocarcinoma, 72, 73, 79 adrenal cortical carcinoma, 72 in, 147 aggressive digital papillary adrenal myelolipoma, 405 PAX-FKHR fusion genes in, 132, 133 adenocarcinoma, 799 adrenocortical clear cell carcinoma, 454 alveolar sarcoma, 559 differential diagnosis, 1035 bilateral adrenal myelolipoma, 405 alveolar soft part sarcoma (ASPS), 2, 30, 1049 ductal adenocarcinoma, 73 cortical adenoma, 64 ASPL-TFE3 gene fusion in, 134 hepatobiliary adenocarcinoma, 72 metastatic adrenal cortical carcinoma, 852 clinical features lung adenocarcinoma, 72 multiple extra-adrenal myelolipoma, 405 in soft tissue, 930–931 metastatic gastrointestinal pheochromocytoma of the adrenal, in visceral sites, 931 adenocarcinoma, 846 758–761 differential diagnosis, 935–936, 1046, 1049

page 1083

© in this web service Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-87409-0 - Modern Soft Tissue Pathology: Tumors and Non-Neoplastic Conditions Edited by Markku Miettinen Index More information

1084 INDEX

alveolar soft part sarcoma (ASPS) (cont.) angiomyolipoma (AML), 63, 64, 531–536, atypical lipoma, 208, 436 genetics, 936 1046. See also perivascular atypical lipomatous tumor (ALT), 83, 447, histogenesis, 935 epithelioid cell tumors 1021, 1059 immunohistochemistry, 934–935 clinical features, 531–532 of subcutis (atypical lipoma), 432–434 pathology, 931–932 differential diagnosis, 536, 1046 atypical tenosynovial giant cell tumor, ultrastructure, 935 genetics and biology, 536 940–961 American Cancer Society, 2, 11 immunohistochemistry, 536 American Joint Committee of Cancer (AJCC) pathology, 532–536 bacillary angiomatosis, 601–602 staging system, 6 angiomyoma. See angioleiomyoma Baker cysts, 35, 314 amputation, role of, 1060 angiomyomatous hamartoma of lymph, banding of chromosome preparations, 107 amputation neuroma (traumatic neuroma), 30, 461 Bartholin’s cyst, 512 32, 662 angiomyxoma, 54, 137 basal cell carcinoma, 839 amyloid tumor (amyloidoma), 976–978 of the pelvis, 80 basal cell nevus syndrome, 551 anaplastic carcinoma, 1030–1031 angiosarcoma, 3, 25, 29, 617, 1039 basement membrane collagen, 182 anaplastic large cell lymphoma (ALCL) breast parenchymal, 628 basement membrane protein, 75–76 differential diagnosis, 1030–1031 cardiovascular, 629–631 collagen type IV (four), 75–76 noncutaneous, 992–994 classic type, 1039 laminin, 75 primary cutaneous, 994 cutaneous, of scalp, 625–626, 631 practical uses, 76 anaplastic lymphoma, 72, 75, 131, 135, 297 deep soft tissue, 628 B-cell lymphoma, 72, 138, 549 ancient schwannoma, 1032 described, 623–625 bcl-2 gene product, 76 androgenic anabolic steroids, 4 differential diagnosis, 504, 600, 633–634 bcl-2 protein, 982 aneurysmal (cellular) fibrous histiocytoma, 229 epithelioid type, 1039 Becker’s nevus/melanosis, 461 angioblastic meningiomas. See meningeal foreign-body associated, 631 Beckwith-Wiedemann syndrome, 551, 896, hemangiopericytoma genetics, 636 897 angiofibroma, cellular, 478, 514–515 germ cell tumor-associated, 631 Bednar tumor (pigmented clinical features, 514 hepatic, 3, 4, 617, 628–629 dermatofibrosarcoma), 675 differential diagnosis, 514 histopathology of, 631–633 benign fibroblastic/myofibroblastic pathology, 514 HRAS/KRAS mutations, 128 proliferations in children angiofibroma, nasopharyngeal, 269–271 immunohistochemistry, 44, 47, 48, 58, 72, angiomatoid (malignant) fibrous clinical features, 269–270 73, 80, 634–636 histiocytoma, 292–297 differential diagnosis, 271 lymphedema-associated, 626–628 calcifying (juvenile) aponeurotic fibroma, pathology, 270–271 nerve sheath tumor-associated, 631 264–265 angiogenesis biology, 574–575 pediatric, 631 calcifying fibrous (pseudo)tumor, 260–261 angiography, 13. See also computed splenic, 628–629 cerebriform fibrous proliferation in tomography angiography (CTA) treatment, 1078 proteus syndrome, 280–281 angiokeratoma, 581–582 well-differentiated, 50 cranial fasciitis, 255–256 angioleiomyoma, 463–466, 478 wide-excision treatment, 1063–1064 fibromatosis coli, 256–258 clinical features, 464 anthracyclines, 1073, 1078 fibrous hamartoma of infancy, 271–273 differential diagnosis, 461 antiangiogenic therapy. See gemcitabine; fibrous umbilical polyp, 258–259 genetics, 466 sorafenib Gardner fibroma, 268–269 pathology, 464–466 APC inactivation/β-catenin activation in Wnt gingival fibromatosis, 258–260 angiolipoma, 402–403 pathway, 143 infantile diffuse fibromatosis, 268 clinical features, 402 appendiceal gastrointestinal stromal tumor infantile (congenital) fibrosarcoma, genetics, 403 (GIST), 497–498 301–302 pathology, 402–403 array-based comparative genomic inflammatory myofibroblastic tumor, angiolymphoid hyperplasia with eosinophilia, hybridization (aCGH), 108, 114, 297–301 590–593 153 juvenile hyaline fibromatosis, 279–280 angiomatoid (malignant) fibrous histiocytoma arsenic (inorganic), 4 lipofibromatosis, 265–268 (AFH), 54, 110, 292–297 arteriovenous hemangioma, 27 myofibroma and myofibromatosis, clinical features, 292–293 of the skin, 583 273–279 differential diagnosis, 293–297, 739 asbestos, and mesothelioma, 816 nasopharyngeal angiofibroma, 269–271 EWSR1 involvement in, 129 Askin’s tumor, 129, 897, 904 neurothekeoma, 285–289 FUS gene involvement, 132 ASPL-TFE3 gene fusion, 134, 934 plexiform fibrohistiocytic tumor (PFHT), genetics, 297 astrocytic glial tumors, 80 289–292 HMGA2 in, 137 ATF-1 gene, 129, 137, 739 recurrent digital fibroma, 261–264 immunohistochemistry, 293 atypical decubital fibroplasia (ischemic benign fibrous histiocytomas pathology, 293 fasciitis), 196–197 (BFHs)(dermatofibroma), angiomatosis, 29, 586 atypical Ewing sarcoma, 900–901 224–234 angiomatous lesions, 21, 25–30. See also atypical fibrous histiocytoma, 232 clinical features, 224–225 hemangiomas; lymphangioma; atypical fibroxanthoma (AFX), 216, 232, differential diagnosis, 228–229 vascular neoplasms 379–380, 509 genetics, 232–234 angiomatous syndromes, 25, 29 clinical features, 379–380 immunohistochemistry, 228 angiomyofibroblastoma, 54, 80, 478, 512. See differential diagnosis, 380, 839 multiple BFHs, 225 also male angiomyofibroblastoma- pathology, 380 pathology, 225 like tumor atypical glomus tumor, 653 recurrences and metastases, 225

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Index 1085

benign fibrous histiocytomas metastatic carcinoma, 841 ovarian carcinoma, 62, 81, 852–854 (BFHs)(dermatofibroma), variants myofibroblastoma, 222, 224 poorly differentiated sinonasal carcinoma, aneurysmal (cellular) fibrous histiocytoma, parenchymal angiosarcoma, 628 750 229 sarcomatoid (spindle cell) carcinoma, primary neuroendocrine carcinoma, 56, atypical fibrous histiocytoma, 232 841–842 834–838 cellular fibrous histiocytoma, 229–230 secretory carcinoma, 136 primary peritoneal/metastatic serous epithelioid fibrous histiocytoma, 230 Budd-Chiari syndrome, 476 papillary carcinoma, 827 granular cell fibrous histiocytoma, 232 Burkitt lymphoma, 836, 989 prostatic carcinoma, 72, 851–852 lipidized fibrous histiocytoma, 230 bursal fluid collection, 34, 35 pulmonary carcinomas, 80 benign lipoma, 441 pulmonary small cell carcinoma, 504, 836, benign lymphangioendothelioma, 609–611 Cajal cell hyperplasia, 501 838 benign mesenchymal tumors, 1 calcific uremic arteriolopathy, 879 salivary gland carcinoma, 62 benign metastasizing leiomyoma, 481 calcifying (juvenile) aponeurotic fibroma, sarcomatoid carcinoma, 301, 379, 522, 798 benign peripheral nerve sheath tumors 264–265 sarcomatoid (spindle cell) carcinoma, (BPNSTs), 13, 32–33 calcifying bursitis. See tumoral calcinosis 841–842 vs. MPNSTs, 33–34 calcifying fibrous (pseudo)tumor (CAFP), sarcomatoid carcinoma (carcinosarcoma), benign rhabdomyoma, 2 260–261 301 benign Triton tumor, 549. See neuromuscular calciphylaxis, 879 sarcomatoid pulmonary carcinoma, hamartoma calcium pyrophosphate deposition disease, 843–846 beta (β)-catenin 946–947 sarcomatoid renal cell carcinoma, 849–851 activation in Wnt pathway, 143 capillary hemangiomas, 27 sarcomatoid squamous cell carcinoma, in fibromatosis, 240 carbon material, synovia and tendon 380, 839 in Gardner fibroma, 211 pathology, 948–950 sclerosing metaplastic carcinoma, 364 bilateral adrenal myelolipoma, 405 carcinoids, 58 secretory breast carcinoma, 136 bilateral vestibular schwannoma, 144–145, 685 carcinomas, 62. See also adenocarcinoma; small cell carcinoma, 57, 904, 1051 biological considerations, 118–122 Merkel cell carcinoma spindle cell carcinomas, 53 multiple, sometimes complex adenoid cystic carcinoma, 1035 squamous carcinoma, 73, 79 abnormalities, 120–122 adrenal cortical carcinoma, 72 thyroid carcinoma, 81, 854 tumor-specific abnormalities adrenocortical clear cell carcinoma, 454 thyroid papillary carcinoma, 131 (translocations), 118–120 basal cell carcinoma, 839 trabecular carcinoma of the skin, 834–838 biopsies breast carcinoma, 62, 81, 136, 143 carcinosarcoma, 520–523, 1078. See also core biopsy, 1057–1058 choroid plexus carcinoma, 142 adenofibroma; adenosarcoma; excisional biopsy, 1058 clear cell renal carcinoma, 564 uterine carcinosarcoma fine-needle aspiration (FNA) biopsy, 1057 colorectal carcinoma, 72, 143 clinical features, 520–521 incisional biopsy, 1058 ductal carcinoma, 58, 80, 1018 genetics/histogenesis, 522–523 in sarcoma resection, 1058 eccrine sweat gland carcinoma, 62 pathology, 521–522 biphasic synovial sarcoma, 51, 54, 62, 63, 69, endometrial carcinoma, 62, 81 cardiac inflammatory myofibroblastic tumors, 74, 780. See also monophasic gastrointestinal carcinoma, 80 298, 300 synovial sarcoma; poorly hepatic carcinoma, 3 cardiac myxoma, 323–325 differentiated synovial sarcoma hepatocellular carcinoma, 72, 846 cardiac rhabdomyoma, 542 immunohistochemistry, 72, 73 infiltrating ductal carcinoma, 136 cardiofaciocutaneous (CFC) syndrome, blastic extramedullary myeloid tumors, 49 intra-abdominal neuroendocrine 128 bone cement, synovia and tendon pathology, carcinoma, 843 cardiovascular angiosarcomas, 629–631 947–948 keratinizing squamous carcinomas, 73 Carney complex, 316, 320, 323. See also bone sarcoma, 143 large cell carcinoma, 842 superficial angiomyxoma (SAM) bone tumors, 1, 11. See also cartilage and laryngeal sarcomatoid carcinoma, 839 Carney-Stratakis syndrome, 758 bone-forming tumors lobular mammary carcinomas, 80, 520 Carney triad, 500–501, 862. See also Borrelia burgdorferi spirochete, 940 medullary thyroid carcinoma, 662, 854 functioning extra-adrenal botryoid-embryonal rhabdomyosarcoma, 508, Merkel cell carcinoma, 834–835 paraganglioma; gastrointestinal 510, 556–559 metastatic adrenal cortical carcinoma, 852 stromal tumor; pulmonary brain tumors, 1, 143, 144–145 metastatic carcinoma, 1, 75, 202, 454, 520, chondroma branchial anlage mixed tumor (ectopic 841, 1042 carotid body paraganglioma, 768 hamartomatous thymoma), metastatic ductal carcinoma, 841 Carrion’s disease, 602–603 802–804 metastatic lobular carcinoma, 841 cartilage and bone-forming tumors, 61, 106 clinical features, 802 metastatic neuroendocrine carcinoma, 56 calciphylaxis, 879 differential diagnosis, 804 metastatic prostatic carcinoma, 851–852 chondroma of soft parts, 862–864 immunohistochemistry/ultrastructure, metastatic pulmonary small cell carcinoma, extraskeletal mesenchymal 804 842–843 chondrosarcoma, 870–871 pathology, 802–804 metastatic renal cell carcinoma, 847–849 extraskeletal myxoid chondrosarcoma, 451, breast carcinoma, 62, 81 metastatic spindle cell carcinoma, 1047 1044, 1051 ETV6-NTRK3 gene fusion in, 136 mucoepidermoid carcinoma of salivary extraskeletal osteosarcoma, 887–889 WT1 overexpression in, 143 glands, 131 fibrodysplasia ossificans progressiva, breast tumors myoepithelial carcinoma, 733, 796 884–885 adenocarcinoma, 72, 79 neuroendocrine carcinoma, 56, 58, 77–79, fibro-osseous pseudotumor of the digits, ductal carcinoma, 58 842–843 887

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1086 INDEX

cartilage and bone-forming tumors (cont.) CD163 (erythrocyte scavenger receptor), children metaplastic bone, 887 67–68, 997, 998, 1002, 1005, 1007 angiosarcoma, 631 myositis ossificans, 11, 881–884 CDK4 (cyclin-dependent kinase 4) benign fibroblastic/myofibroblastic phosphaturic mesenchymal tumor of protein-serine kinase, 139, 441 proliferations mixed connective tissue variant, CDKN1A (p21) gene, 140 angiomatoid (malignant) fibrous 871–875 CDKN2A (cyclin-dependent kinase histiocytoma, 292–297 synovial chondromatosis, 11, 19–20, 21, inhibitor-2A) gene, 141–142 calcifying (juvenile) aponeurotic 37, 864–867 cDNA microarrays (DNA chips/gene arrays), fibroma, 264–265 tophaceous pseudogout, 877–879 153 calcifying fibrous (pseudo)tumor, tumoral calcinosis, 11, 875–877 cell culture/chromosome banding, 105–107 260–261 Castello syndrome, 128 culture initiation and maintenance, cerebriform fibrous proliferation in Castleman’s disease, 586 106–107 proteus syndrome, 280–281 cauda equina/spinal canal paraganglioma, harvest/banding of chromosome cranial fasciitis, 255–256 773 preparations, 107 fibromatosis coli, 256–258 cavernous hemangioma, 464, 580–581 sample preparation, 106 fibrous hamartoma of infancy, 271–273 CD1a, 62, 998, 1002, 1005, 1008 specimen requirements, 105 fibrous umbilical polyp, 258–259 CD2, 992, 997 specimen transport, 105–106 Gardner fibroma, 268–269 CD3, 991, 992, 996 cell cycle markers, 82–84 gingival fibromatosis, 258–260 CD5, 988, 992 cyclin-dependent kinase inhibitors, 84 infantile diffuse fibromatosis, 268 CD7, 992 Ki-67 monoclonal antibody, 82–83 infantile (congenital) fibrosarcoma, CD8, 992 MDM2 (murine double minute), 83–84 301–302 CD10, 985, 987, 988, 989 p53 (TP53) cell cycle regulator, 83 inflammatory myofibroblastic tumor, CD11c, 1008 retinoblastoma gene product (RB), 84 297–301, 504 CD19, 989 cellular angiofibroma, 478, 514–515 juvenile hyaline fibromatosis, 279–280 CD20, 982, 985, 987, 988, 989, 991, 994, 996, cellular blue nevus (CBN), 724–726 lipofibromatosis, 265–268 1006 cellular capillary hemangioma, 402–403 myofibroma and myofibromatosis, CD21, 984, 1008 cellular fibrous histiocytoma, 229–230 273–279 CD22, 989 cellular myxolipomatous tumors, 447 nasopharyngeal angiofibroma, 269–271 CD23, 988, 1008 cellular neurofibroma, 669 neurothekeoma, 285–289 CD25, 997 cellular plexiform schwannoma, 684 plexiform fibrohistiocytic tumor CD30, 991, 992, 994, 996, 1007 cellular schwannoma, 682–683 (PFHT), 289–292 CD31 (platelet endothelium cell adhesion cellular solitary fibrous tumor, 520 recurrent digital fibroma, 261–264 molecule I, PECAM-1), 46–47, central nervous system inflammatory central nervous system lipoma, 398–399 1005 myofibroblastic tumors, 298 Dabska tumor, 617–619 CD34 (hematopoietic progenitor cell central nervous system lipomas, 398–399 gastrointestinal stromal tumor, 500–501 antigen), 47–51 central primitive neuroectodermal tumor juvenile xanthogranuloma, 66, 67, 229, Fli-1 (Freund’s leukemia integration site) (PNET), 142 1002–1004 gene, 51 cerebriform fibrous proliferation in proteus Kaposi-like infantile podoplanin (D 2-40), 50–51 syndrome, 280–281 hemangioendothelioma, 589–590 thrombomodulin (CD141), 51 cervicovaginal myofibroblastoma, 510 leiomyosarcoma, 476–477 vascular endothelial growth factor receptor superficial, 510–511 lipoblastoma, 23, 420–423 3, 51 cervix polyps, 508 liposarcoma, 447 von Willebrand factor, 49–50 CGH (comparative genomic hybridization), lumbosacral/spinal cord-associated lipoma, CD34-positive fibroblasts, 182 108, 114, 153 398 in atypical fibrous histiocytoma, 232 chemical links to sarcomas, 3–4 melanotic neuroectodermal tumor of in BFHs, 225 chemoradiotherapy of non-GIST sarcomas, infancy, 1033 in elastofibroma, 208 1075 vascular malformation syndromes, 612 in nuchal fibrocartilaginous pseudotumor, chemotherapy chloroma, 994–996 211 actinomycin D, 1064 choanal polyps, 271 in pleomorphic fibroma of the skin, adjuvant/neo-adjuvant for non-GIST chondroid lipoma, 397, 414–418 216 sarcomas, 1073–1075 chondroma of soft parts, 862–864 CD35, 1008 cyclophosphamide, 1064 CHOP gene, 110, 120, 129, 137 CD38, 991, 1006 with CyVADIC, 1074 chordoma, 61, 69, 72, 73, 74, 75, 799–801 CD43, 992, 996 dacarbazine, 1062 choroid plexus carcinoma, 142 CD45, 62, 994, 996, 1005, 1008 doxorubicin, 1061, 1064 chromogenic in situ hybridization (CISH), CD56 (NCAM, neural cell adhesion doxorubicin-ifosfamide-dacarbazine 113 molecule), 62, 991, 992 (combination), 1074 chromogranin A, 58 CD57 (myelin associated glycoprotein, Leu7 in extremity/truncal sarcoma, 1061–1063 chromosomal abnormalities, 116–118 HNK1 antibodies), 62–63 histologic subtypes requiring, 1064–1065 chromosome analysis, 107–108 CD63 (NK1C3), 66 ifosfamide, 906, 1062 karyotype, 107–108 CD68 (lysosomal protein of 110 Kd), 67, 998, neoadjuvant chemotherapy, 1063 microscopy/automated image analysis 1007 vincristine, 1064 systems, 107 CD79A, 988, 991 cherry hemangioma, 598 nomenclature, 108 CD117. See KIT Chester-Erdheim disease. See chromosome banding. See cell CD138, 991, 1006 Erdheim-Chester disease culture/chromosome banding

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Index 1087

chronic myelogenous leukemia, 358 CREB1 gene, 129, 132, 297, 740 fat necrosis (FN), 1018 circumscribed storiform collagenoma, CREB3L2 gene, 132, 364 hibernoma, 1019–1021 214–215 Crohn’s disease, 944 lipoblastoma (LB), 1018–1019 cirsoid aneurysm, 583 cryptococcosis, 1018 lipoma, 1017–1018 cisplatin, 1078 crystal deposition disease (focal tumoral myxoid/round cell liposarcoma, classic (chronic) Kaposi’s sarcoma, 636 calcium pyrophosphate dihydrate 1021–1022 classification of soft tissue tumors, 1 [CPPD] deposition), 877–879 myxolipoma (ML), 1018 clear cell renal carcinoma, 564 crystal storing histiocytosis, 1006 spindle cell/pleomorphic lipoma clear cell rhabdomyosarcoma, 562–564 CSPG2-PTK2B fusion gene, 147 (SC/PL), 1019 clear cell sarcoma, 110 culture initiation and maintenance, 106–107 ancillary techniques, 1016–1017 differential diagnosis, 731 cutaneous angiosarcoma, 625–626 cytogenetics, 1017 EWSR1 involvement in, 129 cutaneous focal mucinosis, 317 electron microscopy, 1016 gastrointestinal clear cell sarcoma, cutaneous leiomyomata, 463 flow cytometry, 1016–1017 739–740 cutaneous leiomyosarcomas, 474 immunohistochemistry, 1016 HMGA2 in, 137 cutaneous melanoma, 1 chondro-osseous tumors, 1042–1044 immunohistochemistry, 80 cutaneous myxoid cyst, 314 extraskeletal mesenchymal of kidney, 1046 cutaneous neurofibroma, 669, 672, 675 chondrosarcoma, 870–871, 1044 of soft tissue, 1049 cutaneous pleomorphic liposarcoma, 454 myositis ossificans, 11, 881–884, of tendons and aponeuroses, 736–739 cutaneous Rosai-Dorfman disease (RDD), 1042–1044 clinical features, 736 997–998 (myo)fibroblastic lesions, 1022–1028 differential diagnosis, 737–739 cutaneous sarcomatoid (spindle cell) acral myxoinflammatory fibroblastic genetics, 739 carcinoma, 839 sarcoma, 1026 immunohistochemistry, 737 cyclin-dependent kinase inhibitor-2A adult fibrosarcoma, 364–365, 1026 pathology, 737 (CDKN2A) gene, 141–142 desmoid type fibromatosis, 251, 268, clear cell “sugar” lung tumors, 63 cyclin-dependent kinase inhibitors, 84 1022, 1024 clear cell “sugar” tumor, 531 cyclophosphamide, 1064, 1079. See also infantile myofibroma/fibromatosis, clinodactyly, 238 CyVADIC 1024 clonality assessment using HUMARA, cylindroma, 798 low-grade fibromyxoid sarcoma, 453, 148–149 cylindromatosis syndrome, 797 1028 CNS-based hemangiopericytoma, 336–337 cystic hamartoma, 520 mammary-type myofibroblastoma, COLIA1-PDGFB fusion gene, 137, 358 cystic lesions, 21 1024 collagenous fibroma (desmoplastic cystic masses myxofibrosarcoma, 370–373, fibroblastoma), 211–214 imaging appearances, 34–36 1026–1028 clinical features, 211 types of, 34 nodular fasciitis, 21, 52, 54, 76, differential diagnosis, 212–213 cystic/multicystic peritoneal mesothelioma, 182–189, 1022 genetics, 213–214 811 nerve sheath, neuroectodermal, neural pathology, 211–212 cytogenetic analysis (conventional), 105–108 tumors, 1031–1035 collagens, 75–76, 182 advantages and limitations, 108 ancient schwannoma, 1032 colonic gastrointestinal stromal tumor cell culture/chromosome banding, extracranial meningioma, 1033 (GIST), 497–498 105–107 malignant peripheral nerve sheath colon polyposis, 143 culture initiation and maintenance, tumor, 3, 13, 20, 30, 32–33, 73, 80, colorectal carcinoma, 72, 143 106–107 631, 1035 colorectal gastrointestinal stromal tumor harvest/banding of chromosome melanotic neuroectodermal tumor of (GIST), 493 preparations, 107 infancy, 1033 colorectal leiomyoma, 466–467 sample preparation, 106 myxopapillary ependymoma of soft combined binary ration FISH specimen requirements, 105 tissue, 742, 1033–1035 (COBRA-FISH), 108, 113 specimen transport, 105–106 neurofibroma, 30, 32–33, 48, 62, 669, comparative genomic hybridization (CGH), chromosome analysis, 107–108 1033 108, 114, 153, 252 karyotype, 107–108 schwannoma, 50, 62, 63, 73, 75, 80, computed tomography (CT), 11, 13–15 microscopy/automated image analysis 514, 631, 1031–1032 of hemangiomas, 26 systems, 107 pericytic (perivascular) tumors, 1042 of neurogenic tumors, 30–33 nomenclature, 108 skeletal muscle tumors, 1035–1038 computed tomography angiography (CTA), cytogenetic analysis (molecular), 105, 108–116 alveolar rhabdomyosarcoma, 56, 84, 13 advantages and limitations, 115–116 110, 551, 559–561, 1037–1038 congenital macrogingivae. See gingival fluorescence in situ hybridization (FISH), embryonal rhabdomyosarcoma, 555, fibromatosis 105, 108–115 1036–1037, 1051 congenital nonprogressive hemangioma, probes and hybridization, 110–111 pleomorphic rhabdomyosarcoma, 578 probe strategies, 111–113 561–562, 1038 conventional cytogenetic analysis. See specimen requirements, 108–110 rhabdomyoma, 546, 1035–1036 cytogenetic analysis (conventional) technical variations, 113–115 rhabdomyosarcoma, 30, 51, 52–53, 56, Costello syndrome, 128 cytogenetics/cell culture, 8 550–559, 896, 898, 904, 1036 cotton-fiber granuloma, 973–974 cytology of soft tissues lesions smooth muscle tumors Cowden’s disease, 215 adipocytic lesions, 1017–1022 leiomyosarcoma, 2, 3, 52, 1038–1039 Cowden syndrome, 409 atypical lipomatous tumor/well- so-called fibrohistiocytic tumors, cranial fasciitis, 182, 255–256 differentiated liposarcoma, 1021 1028–1031

© in this web service Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-87409-0 - Modern Soft Tissue Pathology: Tumors and Non-Neoplastic Conditions Edited by Markku Miettinen Index More information

1088 INDEX

cytology of soft tissues lesions (cont.) dendritic reticulum cell sarcoma, 293, 297, differential diagnosis, 1049 deep benign fibrous histiocytoma, 1007–1008 EWSR1 involvement in, 129 1030 fibroblastic reticulum cell sarcoma, WT1 involvement, 129–131 giant cell tumor of tendon sheath, 36, 1008–1010 differential diagnosis 951–952, 1028 interdigitating reticulum cell sarcoma, acral myxoinflammatory fibroblastic undifferentiated pleomorphic sarcoma, 1008 sarcoma, 370, 1026 561, 1030–1031 dendritic reticulum cell sarcoma, 293, 297 adenocarcinoma, 1035 tumors of uncertain differentiation, Denys-Drash syndrome, 143 adult rhabdomyoma, 549 1044–1053 dermatofibroma, 59 aggressive angiomyxoma, 518 alveolar soft part sarcoma, 2, 30, 1049 dermatofibrosarcoma protuberans (DFSP), aggressive digital papillary angiomyolipoma, 63, 64, 531–536, 182, 348–359, 1059. See also adenocarcinoma, 799 1046 pigmented dermatofibrosarcoma alveolar rhabdomyosarcoma, 918, clear cell sarcoma of soft tissue, 1049 protuberans 1037–1038, 1051 desmoplastic small round cell tumor, clinical features, 348–350 alveolar soft part sarcoma, 935–936, 1046, 81, 110, 843, 1051 COLIA1-PDGFB in, 137 1049 epithelioid sarcoma, 733, 790–792, CSPG2-PTK2B fusion gene in, 147 anaplastic carcinoma, 1030–1031 960, 967, 1048 differential diagnosis, 302, 355–358, 669 anaplastic large cell lymphoma, 1030–1031 Ewing sarcoma, 504, 906, 1044, 1047, genetics, 358 angiomatoid (malignant) fibrous 1049 immunohistochemistry, 48, 59, 62, 67, histiocytoma, 293–297 extrarenal malignant rhabdoid tumor, 77–79, 355 angiomyolipoma, 536, 1046 1051–1053 juvenile variant (giant cell fibroblastoma), angiosarcoma, 504, 633–634 extraskeletal myxoid chondrosarcoma, 354–355 atypical fibroxanthoma, 380, 839 451, 1044, 1051 pathology benign fibrous histiocytomas, 228–229 granular cell tumor, 67, 699–703, 1046 fibrosarcomatous transformation, benign lymphangioendothelioma, 611 intramuscular myxoma, 308–309, 1044 350–354 branchial anlage mixed tumor, 804 mixed tumor/myoepithelioma, typical DFSP, 350 cauda equina paraganglioma, 773 792–795, 1044 treatment, new pathogenesis-based, cellular angiofibroma, 514 primitive neuroectodermal tumor, 30, 358–359 cellular blue nevus, 724–726, 737–739 57, 58, 142, 1048–1049 vs. sclerotic fibroma of skin, 215 cellular schwannoma, 683 synovial sarcoma, 2, 11, 19–20, 30, 51, dermatomyofibroma, 216 chondroid lipoma, 416–418, 441 110, 777–787, 904, 1046–1047 desmin chordoma, 801 vascular tumors, 1039–1042 in adult rhabdomyoma, 549 clear cell sarcoma, 731, 1046 angiosarcoma, 3, 25, 29, 617, 1039 in angiomatoid fibrous histiocytoma, 290 collagenous fibroma, 212–213 epithelioid hemangioendothelioma, biology and normal distribution, 53 cranial fasciitis, 255–256 620–623, 1039–1042 in fetal rhabdomyoma, 547 deep benign fibrous histiocytoma, 1030 cytoplasmic chimeric proteins, 135 in neurothekeoma, 290 deep granuloma annulare, 967 CyVADIC (cyclophosphamide, vincristine, in rhabdomyosarcoma, 565, 566 dermatofibrosarcoma protuberans, 302, doxorubicin, dacarbazine), soft tissue tumors, 53–54 355–358 1074 in tenosynovial giant cell tumor, 959 desmoid type fibromatosis, 251, 268, 1022, desmoid fibrosarcoma, 188 1024 Dabska tumor (papillary intralymphatic desmoid leiomyoma, 76 desmoplastic small round cell tumor, 1049 angioendothelioma), 617–619 desmoid tumor, 52, 143, 1060, 1079 ectopic meningioma, 750 dacarbazine, 1062. See also CyVADIC; mesna desmoid type fibromatosis (DTF), 185, 213, embryonal rhabdomyosarcoma, 1051 with doxorubicin, ifosfamide, 243–252, 1022–1024 endometrial stromal sarcoma, 520, 1078 dacarbazine abdominal wall, 244 epithelioid hemangioendothelioma, dactinomycin, 1075, 1079 differential diagnosis, 251, 268, 1022, 1024 1042 dasatinib, for imatinib-resistant GIST, extra-abdominal, 245–246 epithelioid hemangioma, 593 1073 genetics, 252 epithelioid leiomyosarcoma, 1048 dedifferentiated liposarcoma (DDLS), 138, immunohistochemistry, 251 epithelioid sarcoma, 733, 790–792, 960, 378–379, 381, 441–445 intraabdominal wall, 244–245 967, 1048 clinical features, 441 multiple desmoids, 246–247 Ewing sarcoma, 504, 906, 1044, 1047, genetics/cell biology, 444–445 pathogenesis, 251–252 1049 pathology, 442–444 pathology, 247 extrarenal rhabdoid tumor, 921–922 deep benign fibrous histiocytoma (DBFH), treatment, 247 extraskeletal mesenchymal 1030 desmoplastic fibroma, 211–214 chondrosarcoma, 1044 deep fibrous histiocytoma, 335 desmoplastic melanoma, 63, 64, 733–735 extraskeletal myxoid chondrosarcoma, 451, deep granuloma annulare (pseudorheumatoid clinical features, 733 1044, 1051 nodule, necrobiotic granuloma), differential diagnosis, 735 fat necrosis, 1018 966–967 genetics, 735 fetal rhabdomyoma, 547 deep lipomatous tumors (with limited atypia), immunohistochemistry, 735 fibromyxoid sarcoma, low-grade, 364 441 pathology, 733 Gardner fibroma, 269 deep soft tissue angiosarcomas, 628 desmoplastic mesothelioma, 48, 822 gastrointestinal schwannoma, 687 degenerative joint disease (synovia in desmoplastic small cell tumor (DSCT), 896 giant cell angiofibroma, 341 osteoarthritis), 942 desmoplastic small round cell tumor giant cell tumor of tendon sheath, 1028 dendritic cell proliferations (DSRCT), 81, 110, 843, 1051 giant cell tumors of soft parts, 383

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Index 1089

glomus tumor, 655, 1042 pleomorphic lipoma, 409, 441, 1019 ductal carcinoma, 80 granular cell tumor (GCT), 703, 1021, pleomorphic liposarcoma, 1039 of breast, 58 1046, 1049 pleomorphic malignant fibrous differential diagnosis, 1018 hemangiopericytoma, 345 histiocytoma, 378–379 duodenal gangliocytic paraganglioma, hibernoma, 414, 1021 pleomorphic rhabdomyosarcoma, 770–773 infantile (congenital) fibrosarcoma, 302 1038 duodenal gastrointestinal stromal tumor inflammatory MFH, 381–383 plexiform fibrohistiocytic tumor, 290 (GIST), 493, 497 inflammatory myofibroblastic tumor, 301 proliferative fasciitis, 1022 Dupuytren’s contracture. See palmar and intramuscular myxoma, 309, 451, 518, proliferative myositis, 196, 1022 plantar fibromatosis 1021–1022, 1028 psammomatous melanotic schwannoma, dysgerminoma, 854–855 juxtaarticular myxoma, 309 689–691, 731 dysphagia, 467 Kaposi’s sarcoma, 633, 641–642 recurrent digital fibroma, 264 leiomyosarcoma, 297, 301, 504, 1039, reticulohistiocytoma, 1005 ear (external) meningioma, 748–749 1047, 1078 retroperitoneal fibrosis, 202 EBV. See Epstein-Barr virus (EBV) lipoblastoma, 1019 rhabdomyoma, 906, 1021 EBV-associated myofibroblastic lipofibromatosis, 267–268, 290, 421 rhabdomyosarcoma, 444, 569, 906, 1049, proliferations, 297 lipoma, 396, 448, 1017, 1019 1053 eccrine acrospiroma (hidradenoma), 131, low-grade myxofibrosarcoma, 1019, 1028 round cell liposarcoma, 1021–1022 796–797 lymphangioma, 606–607 schwannoma, 387, 504, 1019, 1024, 1048 eccrine spiradenoma, 797–798 male angiomyofibroblastoma-like tumor, sclerosing epithelioid fibrosarcoma, 364 eccrine sweat gland carcinoma, 62 514 sinonasal hemangiopericytoma, 656–658 ectomesenchymoma, 565–566, 904 malignant glandular MPNSTs, 712 small cell carcinoma, 1051 ectopic glial tissue (nasal glioma/soft tissue malignant melanoma, 1053 small round cell desmoplastic tumor, 918 gliomatosis), 741–742 mammary-type myofibroblastoma, 1024 small round cell tumor, 896 ectopic hamartomatous thymoma (branchial melanoma, 379, 1030–1031 soft tissue lipoma, 396 anlage mixed tumor), 802–804 Merkel cell carcinoma, 838, 842 solitary fibrous tumor, 337–341 ectopic meningioma in paranasal soft tissue, mesodermal stromal polyp, 510 spindle cell lipoma, 409, 1019, 1021–1022 746–748 metastatic adenocarcinoma, 1048 superficial acral fibromyxoma, 317, ectopic meningothelial elements of scalp, metastatic carcinoma, 1042 321–323 745–746 metastatic malignant melanoma, 1042, superficial angiomyxoma, 317–320, 518 elastofibroma, 21, 207–208 1049 synovial sarcoma, 342, 787, 1035, 1047, electron microscopy, 9 metastatic melanoma, 731–733, 1048 1048 elephantiasis gingivae. See gingival metastatic spindle cell carcinoma, 1047 tendon sheath fibromas, 214 fibromatosis mixed tumor/myoepithelioma, 795, 1044, tenosynovial giant cell tumor, 960 EMA. See epithelial membrane antigen 1051 atypical/malignant, 961 (EMA) monophasic spindle cell synovial sarcoma, undifferentiated pleomorphic sarcoma, embryonal rhabdomyosarcoma (ERMS), 555, 1026 1030–1031 1036–1037, 1051. See also MPNSTs, 711, 1026, 1035, 1047, 1049 well-differentiated liposarcoma, 396, 414, botryoid-embryonal multicystic peritoneal mesothelioma, 813 441, 448, 451, 1017 rhabdomyosarcoma myoepithelioma, 795 Wilms’ tumor, 906 clinical features, 555 myofibroma/myofibromatosis, 279 differentiated myogenic sarcoma, 379 differential diagnosis, 301 myositis ossificans, 1042 diffuse angiokeratoma, 581 genetics, 555 myxofibrosarcoma, 372, 444, 451, diffuse large B-cell lymphoma, 988 HRAS/KRAS mutations, 128 1021–1022 diffuse lipomatosis of extremities, 401 immunohistochemistry, 59, 84 myxoid liposarcoma, 309, 414, 421, 451, diffuse malignant mesothelioma, 816 MYCN amplification in, 138–139 1019, 1021–1022 diffuse neurofibroma, 675 encephalocraniocutaneous lipomatosis, myxolipoma, 1018 diffuse symmetric lipomatosis, 401 401 myxopapillary ependymoma of soft tissue, diffuse villous synovial hyperplasia, 960 endemic Kaposi’s sarcoma, 637 1035 digital mucoid cyst, 314 endometrial carcinoma, 62, 81 nasopharyngeal angiofibroma, 271 dioxin contaminants, 3 endometrial stromal sarcoma (ESS), 518–520, neck paragangliomas, 769–770 disseminated xanthoma, 972 1078 neuroblastoma, 906 distal interphalangeal joint ganglion cysts, clinical features, 519 neurofibroma, 677–678 314 differential diagnosis, 520, 1078 neurothekeoma, 289 DNA amplification, 138 genetics, 520 nodular fasciitis, 188–189, 251, 255–256, DNA FISH probes, 108 immunohistochemistry, 77, 80 1022, 1024, 1030 DNA mutations, 127 pathology, 519–520 nodular hidradenoma, 1048 DNA viruses, 3 endometriosis, 524–526, 813 organizing hematoma, 966 docetaxel, 1077 endothelial biology, 574–575 ossifying fibromyxoid tumor, 331 doxorubicin, 1061, 1074, 1075, 1077, 1079. endothelial/multispecific markers, 44–47 palmar and plantar fibromatosis, 240 See also CyVADIC; mesna with Enneking staging system, 6, 18 parachordoma, 796 doxorubicin, ifosfamide, eosinophilia-myalgia syndrome, 199 perineurioma, 697–699 dacarbazine eosinophilic fasciitis, 199 peritoneal leiomyomatosis, 483 doxorubicin-ifosfamide-dacarbazine eosinophilic granuloma. See Langerhans cell pleomorphic hyalinizing angiectatic chemotherapy, 1074 histiocytosis tumor, 387 ductal adenocarcinomas, 73 ependymal glial tumors, 80

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1090 INDEX

ependymoma, 80 esthesioneuroblastomas, 57 panniculitis-like T-cell lymphoma, abdominal, 744 estrogen-receptor positive smooth muscle 991–992 myxopapillary, 742 tumors, 477–478 primary cutaneous anaplastic large cell epidemic Kaposi’s sarcoma, 636–637 etiology of soft tissue tumors, 3 lymphoma, 994 epidemiology of soft tissue tumors, 2–3, 11 ETV6-NTRK3 fusion gene, 136, 302 extrarenal malignant rhabdoid tumor epidermal inclusion cyst rupture, histiocytic European Organisation for the Research and (EMRT), 1051–1053 reaction, 972–973 Treatment of Cancer (EORTC), extrarenal rhabdoid tumor, 918–922 epimutation, 127 1078 clinical features, 918–919 epirubicin, 1074 evaluation of soft tissue specimens, 7–8 cytogenics, 921 epithelial cells/carcinomas, 62 gross photography, 8 definition, 918 epithelial membrane antigen (EMA), 293, 456, grossing, 7–8 differential diagnosis, 921–922 473, 565, 695, 777, 871, 920 open biopsy/resection specimen, 7 electron microscopy, 919 epithelial neuroendocrine tumors, 57, 59 reporting, 8 gross/microscopic description, 919 epithelial tumors everolimus, 504 history, 918 POU 5F1 transcription factor in, 131 Ewing sarcoma (ES), 30, 559, 897–906, immunohistochemistry, 920–921 of unknown histogenesis 1048–1049, 1078. See also atypical terminology/synonyms, 918 epithelioid sarcoma, 787–792 Ewing sarcoma; peripheral extrarenal Wilms’ tumor, 918 large cell “proximal type” epithelioid primitive neuroectodermal tumor extraskeletal chondroma, 862 sarcoma, 792 clinical features, 898–899 extraskeletal chondrosarcoma, 11, 36 synovial sarcoma, 2, 11, 19–20, 30, 51, definition, 897 extraskeletal Ewing sarcoma, 30 110, 777–787, 904, 1046–1047 differential diagnosis, 504, 906, 1044, extraskeletal mesenchymal chondrosarcoma epithelioid angiosarcoma, 631–633 1049 (EMC), 870–871, 1044 epithelioid fibrous histiocytoma, 230 electron microscopy, 901–903 clinical features, 870 epithelioid gastric GIST, 496 genetics, 904–906 differential diagnosis, 1044 epithelioid granuloma, 944 gross features, 899 imaging, 870–871 epithelioid hemangioendothelioma (EHE), history, 897 pathology, 871 620–623, 1039–1042 immunohistochemistry, 51, 57, 59, 77–79, extraskeletal myxoid chondrosarcoma clinical features, 620 80, 903–904 (ESMC), 110, 867–870, 1051 genetics, 623 microscopic features, 899–901 clinical features, 867–868 immunohistochemistry, 47, 48, 50, 72, 73 prognosis/outcome, 906 differential diagnosis, 451, 1044, 1051 pathology, 621–623 terminology/synonyms, 897 EWSR1 in, 129 treatment, 1078 EWSR1 gene, 77, 110, 119, 129–132, 146, genetics, 870 epithelioid hemangioma (EH), 590–593 731, 870 imaging, 868 clinical features, 592 EWSR1-DDIT3 (EWS-CHOP) fusion, immunohistochemistry, 57, 61 differential diagnosis, 593 132 NR4A3 in, 131 of the heart, 593 EWSR1-ERG fusion gene, 146 pathology, 868–870 pathology, 592–593 EWSR1-FLI1 fusion gene, 146 extraskeletal osteosarcoma (extraskeletal epithelioid histiocytes granulomas, 66 EWSR1-NR4A3 gene fusion protein, 131 osteogenic sarcoma, extraosseous epithelioid leiomyoma, 479, 539 external beam radiation therapy (EBRT), osteosarcoma), 81, 887–889 epithelioid leiomyosarcoma, 1048 1061 extremity angiosarcoma, 1063 epithelioid malignant peripheral nerve sheath external ear meningioma, 748–749 extremity-based well-differentiated tumor (MPNST), 712–714 extra-abdominal desmoids, 245–246 liposarcoma, 435 epithelioid peripheral nerve sheath tumor, extracranial meningioma (EM), 1033 extremity sarcoma, 1059 691 extragastrointestinal gastrointestinal stromal extremity/truncal radiation, 1061 epithelioid sarcoma (ES), 787–792, 1047–1048 tumor (EGIST), 499–500 clinical features, 787–788 extramedullary myeloid tumor, 66, 80, Fabry disease, 581, 582 differential diagnosis, 733, 790–792, 960, 994–996 FACIT-collagens, 182 967, 1048 extranodal B-cell lymphomas Factor VIII-related antigen. See also von genetics, 792 Burkitt lymphoma, 989 Willebrand factor (vWF, Factor immunohistochemistry, 49, 51, 69, 72, 73, diffuse large B-cell lymphoma, 988 VIII-related antigen) 74, 75, 77, 790 extranodal marginal zone lymphoma, Factor XIIIa (FXIIIa) enzyme protein, 67 pathology, 788–790 987–988 familial adenomatous polyposis (FAP) vs. palmar/plantar fibromatosis, 240 follicular lymphoma, 985–987 syndrome, 211, 243, 244, 269. See epithelioid schwannoma, 684 intravascular B-cell lymphoma, 988–989 also Gardner syndrome epithelioid smooth muscle tumors of the plasmacytoma/plasmablastic B-cell of the colon, 143 gynecologic tract, 479 lymphoma, 989–991 desmoid tumors, 143, 246–247, 251 Epstein-Barr virus (EBV), 3 precursor B-cell lymphoblastic lymphoma, familial gastrointestinal stromal tumor (GIST) Epstein-Barr virus-associated 988–989 syndrome, 501 leiomyosarcoma, 485–486 extranodal lymphoid tumors, 1 familial GT (glomuvenous malformation), Erdheim-Chester disease, 998–1000 extranodal marginal zone lymphoma, 987–988 655–656 ERG gene, 119, 129, 146, 147, 905, 917 extranodal T-cell (and related) lymphomas familial infiltrative fibromatosis (FIF), 143 eruptive xanthoma, 972 nasal-type natural killer cell/T-cell familial paraganglioma (PGL) syndromes, esophageal gastrointestinal stroma tumor (NK/T)-cell lymphoma, 992 757 (GIST), 493 noncutaneous anaplastic large cell fasciectomy for plantar/palmar fibromatosis, esophageal leiomyomatosis, 467 lymphoma, 992–994 239

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Index 1091

fasciitis ossificans, 185 fibromatosis, 21, 37, 204, 335 Fli-1 (Freund’s leukemia integration site) fat atrophy (lipodystrophy), 423 description, 238 gene, 51 fat necrosis/lipogranuloma, 423, 441, desmoid fibromatosis, 243–252 florid endothelial hyperplasia, 600 1018 abdominal wall, 244 florid lobular capillary hemangioma, 579 FDG PET, 13, 34 differential diagnosis, 251 florid vascular proliferation with intestinal female genital stromal tumors, 54, 80. See also extra-abdominal, 245–246 intussusception, 603 angiomyofibroblastoma; genetics, 252 fluorescence in situ hybridization (FISH), angiomyxoma, of the pelvis; immunohistochemistry, 251 108–115. See also array-based endometrial stromal sarcoma intraabdominal wall, 244–245 comparative genomic female leiomyomas of external genitalia and multiple desmoids, 246–247 hybridization; chromogenic in situ uterus, 478 pathogenesis, 251–252 hybridization; combined binary FET family (RNA-binding proteins), 129 pathology, 247 ration FISH; comparative genomic fetal rhabdomyoma, 511, 546–548 treatment, 247 hybridization; DNA FISH probes; clinical features, 546–547 familial infiltrative fibromatosis (FIF), 143 FICTION method; multicolor differential diagnosis, 547 neurofibromatosis type 1 gene (NF1), FISH; reverse genetics, 548 143–144 transcriptase-polymerase chain histologic features, 547 neurofibromatosis type 2 gene (NF2), reaction (RT-PCR) analyses immunohistochemistry, 547–548 144–145 with break-apart arrangement probes, 145 ultrastructure, 547 palmar and plantar fibromatosis, 238–243 probe strategies, 111–113 FFPE (fresh and formalin-fixed penile fibromatosis, 243 probes and hybridization, 110–111 paraffin-embedded tissues), 153 subcutaneous pseudosarcomatous RT-PCR assay discrepancies with, 147 fibroadenoma (epithelial), 58, 137 fibromatosis, 21, 52, 54, 76, specimen requirements, 108–110 fibroblast growth factors/receptors, 182 182–189, 1022 technical variations, 113–115 fibroblastic reticulum cell sarcoma, fibromatosis colli, 256–258 focal cutaneous mucinosis, 320–321 1008–1010 fibromyxoid sarcoma, low-grade, 359–364 focal fluid collection, 34, 35 fibroblastic tumors, 77 clinical features, 359 focal myositis, 550 immunohistochemistry, 48 differential diagnosis, 364 focal papillary endothelial hyperplasia, 601 molecular mechanisms, 182 genetics, 364 focal tumoral calcium pyrophosphate fibroblasts immunohistochemistry, 360 dihydrate (CPPD) deposition biology of, 181–182 pathology, 359–360 (crystal deposition disease), definition, 181 fibromyxosarcoma, 48, 75, 364 877–879 subsets fibro-osseous pseudotumor of the digits follicular lymphoid hyperplasia, 982 CD34-positive fibroblasts, 182 (FOPD), 887 follicular lymphoma (follicular center cell collagens, 182 fibrosarcoma, 3, 52, 77, 335. See also lymphoma), 76, 985–987 growth factors/receptors, 182 dermatofibrosarcoma protuberans foot (pedal) hemangioma, 585 myofibroblasts, 181–182 acral myxoinflammatory fibroblastic Fordyce angiokeratoma, 581 fibrodysplasia ossificans progressiva (FOP), sarcoma, 365–370 foreign-body associated angiosarcoma, 631 884–885 adult fibrosarcoma, 364–365 Fournier’s gangrene, 200 clinical features, 884 dermatofibrosarcoma protuberans (DFSP), FOX gene subfamilies, 133 genetics/biology, 885 348–359 Frasier syndrome, 143 imaging, 884 differential diagnosis, 444, 787 fresh and formalin-fixed paraffin-embedded pathology, 884–885 fibromyxoid sarcoma, low-grade, 359–364 tissues (FFPE), 153 fibrohistiocytic tumor neurothekeoma, 66 infantile (congenital) fibrosarcoma, 111, Freund’s leukemia integration site (Fli-1) fibrolipoma, 208 301–302 gene, 51 fibrolipomatous hamartoma of the nerve, myofibroblastic sarcoma, 364–365 frozen tissue specimens, 8 399–401 myxofibrosarcoma, 370–373 functioning extra-adrenal paraganglioma, 862 fibromas. See also benign fibrous histiocytomas sclerosing epithelioid fibrosarcoma, 364 fungal synovitis, 946 calcifying aponeurotic fibroma, 264–265 fibrous dysplasia (Mazabraud syndrome), 20 FUS-CREB3L2 chimeric protein, 132 collagenous fibroma, 211–214 fibrous hamartoma of infancy, 271–273 FUS-DDIT3 (FUS-CHOP) gene fusion, 132, dermatomyofibroma, 216 fibrous histiocytoma, 67, 77. See also sclerotic 453 elastofibroma, 21, 207–208 benign fibrous histiocytoma FUS-ERG gene fusion, 129 Gardner fibroma, 210, 211, 268–269 of tendon sheath, 951–952 FUS gene, 132 myofibroblastoma of the breast, 222–224 vs. palmar/plantar fibromatosis, 240 fusion genes/transcripts. See also molecular nuchal fibrocartilaginous pseudotumor, fibrous mesothelioma. See solitary fibrous genetics of soft tissue tumors 211 tumors (SFTs) COLIA1-PDGFB, 137 nuchal-type fibroma, 208–211 fibrous umbilical polyp, 258–259 detection of (and transcripts ), 145–146 of oral cavity fibroxanthoma, 951–952 ETV6-NTRK3, 136 giant cell, 220 FICTION method (fluorescence EWSR1-ERG, 146 irritation, 219–220 immunophenotyping, and EWSR1-FLI1, 146 palisaded myofibroblastoma, 220–222 interphase cytogenics as a tool for EWSR1 in, 129–132 pleomorphic fibroma, 215–216 the investigation of neoplasms), 113 JAZF1-JJAZ1, 146 of skin filarial infection-associated lymphedema, pathologic, 128–129 pleomorphic, 215–216 627–628 PAX-FKHR, 132, 133 sclerotic, 214–215 fine-needle aspiration (FNA) biopsy, 1057 PAX-FOXO1A, 146 of tendon sheath, 52, 214 fixatives for tissue specimens, 8–9 PAX3-AFX, 147

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1092 INDEX

fusion genes/transcripts (cont.) duodenal/small intestine GISTs, 493, fibrodysplasia ossificans progressiva, 885 PAX3-FOXO1A, 147 497 fibromyxoid sarcoma, low-grade, 364 PAX3-NCOA1, 147 epithelioid gastric GIST, 496 fibrous hamartoma of infancy, 273 PAX7-FOXO1A, 147 esophageal GISTs, 493 gastrointestinal leiomyoma, 469 prognostic value, 146–147 extragastrointestinal GISTs, 499–500 gingival fibromatosis, 260 SS18-SSX, 133–134, 146, 147 gastric GIST, 493–497 hibernoma, 414 SS18-SSX2, 147 hypercellular spindle cell GIST, 494 infantile (congenital) fibrosarcoma, 302 SS18L1/SSX1, 147 rectal GIST, 499 inflammatory myofibroblastic tumor, 301 tumor-specific transcript determination, sarcomatous spindle cell GIST, intramuscular myxoma, 309 146 494–496 juvenile hyaline fibromatosis, 279 markers juxtaarticular myxoma, 312 gadolinium-associated fibrosis, 976 CD34, 502 lipoblastoma, 421 ganglion cysts, 34, 35, 312–314 keratin/neural, 502 lipomatoses, 402 clinical features, 312–314 KIT, 501–502 melanocytic neuroectodermal tumor of distal interphalangeal joint, 314 muscle cell, 502 infancy, 741 pathology, 314 muscle cell markers, 502 Merkel cell carcinoma, 838 ganglioneuromatosis, 662–664 in neurofibromatosis 1 patients, 501 mixed tumor/myoepithelioma, 795 Gardner fibroma, 210, 211, 268–269 palliative surgery for, 1072 MPNST, 711 Gardner syndrome, 210, 211, 243, 244, 251 PDGFRA mutations in, 491 myoepithelioma, 795 gastric glomus tumor, 653 sunitinib/sunitinib malate treatment, 504, myositis ossificans, 884 gastrointestinal carcinoma, 80 1066, 1071 myxofibrosarcoma, 372–373 gastrointestinal clear cell sarcoma, 739–740 surgical management of, 1066 myxoid liposarcoma, 451–453 gastrointestinal glomus tumor, 648 treatment of, 504, 1066 myxopapillary ependymoma, 742 gastrointestinal leiomyoma, 76. See also surgical, for advanced GIST, 1066 neuroblastoma, 912–914 esophageal leiomyomatosis; tyrosine-kinase inhibitor resistant, nodular fasciitis, 189 intramural leiomyoma; leiomyoma 1072–1073 palmar and plantar fibromatosis, 240–243 of muscularis mucosae; gastrointestinal tract lymphangioma, 606 PEComas, 542 leiomyomatosis gemcitabine, 1077, 1078 pleomorphic lipoma, 409 gastrointestinal leiomyosarcoma, 475–476 gene transfer assays, 127 pleomorphic liposarcoma, 456 gastrointestinal lipoma, 399 genetic events/molecular pathologic pleomorphic malignant fibrous gastrointestinal neurofibromatosis, 679 approaches, 105 histiocytoma, 378 gastrointestinal schwannoma, 686–687 “genetic revolution,” 127 plexiform fibrohistiocytic tumor, 292 gastrointestinal stromal tumor (GIST), 48, 54, genetics. See also molecular genetics of soft proliferative myositis, 196 62, 80, 467, 493–497. See also tissue tumors sclerosing epithelioid fibrosarcoma, 364 appendiceal gastrointestinal stromal acral myxoinflammatory fibroblastic small round cell tumors, 896–897 tumor; epithelioid gastric GIST; sarcoma, 370 soft tissue lipoma, 396–397 hypercellular spindle cell GIST; adult granulosa cell tumor, 524 solitary fibrous tumor, 341 palisaded-vacuolated GIST; adult rhabdomyoma, 549 spindle cell lipoma, 409 sarcomatous spindle cell GIST; aggressive angiomyxoma, 518 superficial angiomyxoma, 320 sclerosing spindle cell GIST alveolar rhabdomyosarcoma, 561 synovial chondromatosis, 867 adjuvant therapy for, 1072 alveolar soft part sarcoma, 936 synovial sarcoma, 785–787 CD34 markers, 502 angioleiomyoma, 466 tendon sheath fibromas, 214 CDKN2A in, 141 angiolipoma, 403 tenosynovial giant cell tumor, 959–960 in children/Carney triad, 500–501, 862 angiomatoid (malignant) fibrous tophaceous pseudogout, 879 clinical features/prognosis, 492–493 histiocytoma, 297 tumoral calcinosis, 877 dasatinib treatment for, 1073 angiomyolipoma, 536 well-differentiated liposarcoma, 441 defined, 491 angiosarcoma, 636 genital (vaginal) rhabdomyoma, 510, 511–512, differential diagnosis, 297, 301, 444, 475, benign fibrous histiocytomas, 232–234 549 503–504 carcinosarcoma, 522–523 genital stromal tumors, female, 54 epidemiology, 491 cardiac myxoma, 325 genitourinary tract leiomyoma, 470 familial GIST syndrome, 501 chondroid lipoma, 418 germ cell tumor–associated angiosarcoma, 631 genetic changes (other than chondroma of soft parts, 864 giant cell angiofibroma (variant of solitary KIT/PDGFRA), 503 collagenous fibroma, 213–214 fibrous tumor), 341–342 histogenesis/pathogenesis, 492 dedifferentiated liposarcoma, 444–445 giant cell fibroblastoma (juvenile variant of history/terminology, 491 desmoid fibromatosis, 252 DFSP), 302, 354–355 HRAS/KRAS mutations, 128 desmoplastic melanoma, 735 giant cell fibroma of the oral cavity, 220 imatinib treatment, 81, 491, 1066, elastofibroma, 208 giant cell hyaline angiopathy (pulse 1070–1071 embryonal rhabdomyosarcoma, 555 granuloma), 967–968 immunohistochemistry, 72, 76, 80, 81, endometrial stromal sarcoma, 520 giant cell malignant fibrous histiocytoma 501 epithelioid hemangioendothelioma, 623 (MFH), 383–384 KIT/PDGFRA mutations in, 136, 491, epithelioid sarcoma, 792 giant cell tumor of tendon sheath (GCTTS), 492, 502–503 Ewing sarcoma, 904–906 36, 951–952, 1028 locations, 491–492 extraskeletal myxoid chondrosarcoma, 870 giant cell tumors of soft parts, 383 appendiceal/colonic GIST, 497–498 extraskeletal osteosarcoma, 889 gigantism of the gingiva. See gingival colorectal GISTs, 493 fetal rhabdomyoma, 548 fibromatosis

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Index 1093

gingival fibromatosis, 258–260 superficial vascular pseudoaneurysm, Henderson-Jones disease, 864–867 gingival granular cell tumor of newborn, 603–605 hepatic angiomyolipoma, 531 703–704 verruga peruana, 602–603 hepatic angiosarcoma, 3, 4, 617, 628–629 GIST. See gastrointestinal stromal tumor hemangiomas, 11, 25 hepatic carcinoma, 3 glandular cystitis, 401 acquired elastotic hemangioma, 598 hepatic epithelioid hemangioendothelioma, Gleevec/Glivec, 136–137, 358, 504 angiogenesis/endothelia biology, 574–575 620 Gli-1 gene, 441 angiokeratoma, 581–582 hepatobiliary adenocarcinomas, 72 glial hamartoma. See ectopic glial tissue (nasal angiomatosis, 586 hepatocellular carcinoma, 72, 846 glioma/soft tissue gliomatosis) arteriovenous hemangioma, 27 hereditary gingival fibromatosis (HGF). See glomangiomyoma, 653 of the skin, 583 gingival fibromatosis glomeruloid hemangioma, 586–587 capillary hemangioma, 27, 581 hereditary leiomyomatosis and renal cell glomus tumor (GT), 49, 52–53, 75, 648–656, cavernous hemangioma, 580–581 cancer (HLRCC), 463 1042 cellular capillary hemangioma, 402–403 hernia sac lipoma, 396, 399 atypical and malignant GTs, 653 cherry hemangioma, 598 heterologous carcinosarcoma, 522 clinical features, 648–650 classification of, 575 heterotopic bone formation (myositis differential diagnosis, 655 congenital nonprogressive hemangioma, ossificans), 11, 881–884 familial GT (glomuvenous malformation), 578 heterotopic neural nodule, 745–746 655–656 CT evaluation, 26 HHF-35 (muscle-specific actin), 52 glomus bodies, 648 epithelioid hemangioma, 590–593 hibernoma (H), 23, 30, 413–414, 1019–1021 immunohistochemistry, 54, 654–655 florid lobular capillary hemangioma, 579 hidradenoma (eccrine acrospiroma), 131, pathology, 650–653 glomeruloid hemangioma, 586–587 796–797 ultrastructure, 654–655 hobnail hemangioma, 587–589 highly active antiretroviral therapy (HAART), glomuvenous malformation (familial GT), immunohistochemistry, 47 401, 423 655–656 in infancy and childhood, 25–26 hip stone disease. See tumoral calcinosis gout, synovial, 946 intramuscular hemangioma, 585–586 histiocyte-related cells, 62 gouty arthritis, 946 juvenile capillary hemangioma, 575–578 histiocytic infiltrates, 1 grading of sarcomas, 4–5. See also Pediatric Kaposiform hemangioendothelioma, histiocytic markers, 66–68 Oncology grading system 589–590 AACT/AAT, 67 limitations, 4–5 lobular capillary hemangioma, 578–579 CD68, 67 systems, 4 microvenular hemangioma, 598 CD163, 67–68 granular cell fibrous histiocytoma, 232 MRI evaluation, 26–27 Factor XIIIa, 67 granular cell tumor (GCT), 67, 699–703, 1046 non-involuting congenital hemangioma, lysozyme (muramidase), 66–67 clinical features, 699–700 575, 578 histiocytic proliferations differential diagnosis, 703, 1021, 1046, rapidly involuting congenital hemangioma, crystal storing histiocytosis, 1006 1049 575, 578 Erdheim-Chester disease, 998–1000 immunohistochemistry/ultrastructure, spindle cell hemangioma, 402–403, histiocytic sarcoma, 66, 67, 68, 1006 700–703 593–598 juvenile xanthogranuloma, 66, 67, 229, pathology, 700 synovial involvement, 27–29 1002–1004 granulomatous synovitis, 944–946 telangiectasia, 598–600 Langerhans cell histiocytosis, 67, granulosa cell tumor, 479 vascular malformations/syndromes 1000–1002 adults, 523–524 featuring, 612 multicentric reticulohistiocytosis, gross photography (evaluation method), 8 venous hemangioma, 575, 583–585 1005–1006 grossing (evaluation method), 7–8 verrucous hemangioma, 582–583 reticulohistiocytoma, 1004–1005 GTW (Giemsa-trypsin-Wright) banding, vs. telangiectasia, 575 Rosai-Dorfman disease, 997–998 107 vs. vascular malformation, 575 histiocytic sarcoma, 66, 67, 68, 1006 hemangiopericytoma (HPC), 25, 29, 52, 335, histiocytic tumors, 66 hamartoma with ectopic meningothelial 342–343, 345, 519. See also histogenesis of soft tissue tumors, 1–2 elements, 745–746 lipomatous hemangiopericytoma histoplasmosis, 202 harvest/banding of chromosome preparations, clinical features, 343 HIV-AIDS, 401, 423 107 differential diagnosis, 337–341, 345, 444, HMB-45 monoclonal antibody, 63 heart lipomas, 399 514 HMGA transcription factor genes, heat shock protein inhibitors, 504 genetics, 345 rearrangement of, 137–138 heavy caldesmon (HCD), 54 immunohistochemistry/ultrastructure, HMGA1 gene, 137, 138 hemangioendothelioma, 25, 29, 617 52–53, 77–79, 343–345 HMGA2 gene, 120, 137, 138, 139, 396–397, epithelioid hemangioendothelioma, pathology, 343 518, 864 620–623 hematolymphatic tumors. See extranodal hobnail hemangioma (targetoid hemosiderotic retiform hemangioendothelioma, 619 lymphoid tumors; histiocytic hemangioma), 587–589 hemangioma-like reactive vascular infiltrates; lymphoma Hodgkin’s disease, 75, 383 proliferations hematomas, 34 host factors, 4 acroangiodermatitis, 603 liquified, 35–36 HRAS proto-oncogene, 128 bacillary angiomatosis, 601–602 hematopoietic tumors, 520 Human Genome Organisation (HUGO) florid vascular proliferation with intestinal hemophilia joint, 942 Nomenclature Committee, 110 intussusception, 603 hemosiderotic fibrohistiocytic lipomatous Human Genome Project, 153 papillary endothelial hyperplasia, 600–601 lesion/tumor (HFLL), 420 human herpes virus 8 (HHV8), 3, 617, 637, reactive angioendotheliomatosis, 603 hemosiderotic synovitis, 942–944, 960 641

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1094 INDEX

HUMARA basement membrane proteins, 75–76 S100 protein/multispecific neural markers, BFHs and, 232 biphasic synovial sarcoma, 72, 73 59–63 clonality assessment using, 148–149 branchial anlage mixed tumor, 804 sarcomatoid carcinoma, 841 locus analysis, 208 cadherins, 76–77 schwannoma (conventional), 680–682 palmar fibromatosis and, 243, 252 calretinin, 79 small round cell desmoplastic tumor, 916 hyaline vascular Castleman disease, 982–984 CD10, 77 synovial sarcoma, 75, 76, 77–79, 783–785 hypercellular spindle cell GIST, 494 CD34, 47–51 technical considerations, 84–86 hyperthermia for non-GIST sarcomas, CD99, 77–79 epitope retrieval, 85 1075 cell cycle markers, 82–84 fixation, 84–85 hypertrophic gingivitis. See gingival chordoma, 61, 69, 72, 73, 74, 75 immunostain interpretation, 86 fibromatosis dermatofibrosarcoma protuberans, 48, 59, optimization, automation, and hypopharyngeal well-differentiated 62, 67, 77–79, 355 standardization, 85–86 liposarcoma, 435 desmoid fibromatosis, 251 tenosynovial giant cell tumor, 959 desmoplastic melanoma, 735 vimentin, 81 iatrogenic immunosuppression-associated endothelial/multispecific markers, well-differentiated liposarcoma, 438–441 Kaposi’s sarcoma, 637 44–47 Wilms’ tumor protein, 81–82 ICC hyperplasia, 136 epithelial membrane antigen (MUC1), infantile diffuse fibromatosis, 268 ifosfamide, 906, 1062, 1074, 1078, 1079. See 74–75 infantile digital fibromatosis. See recurrent also mesna with doxorubicin, epithelioid hemangioendothelioma, 623 digital fibroma ifosfamide, dacarbazine epithelioid sarcoma, 49, 51, 69, 72, 73, 74, infantile (congenital) fibrosarcoma, 111, imaging 75, 77, 790 301–302 phosphaturic mesenchymal tumor of estrogen/progesterone receptors, 79–80 clinical features, 301–302 mixed connective tissue variant, Ewing sarcoma, 51, 57, 59, 77–79, 80, differential diagnosis, 302 872–873 903–904 ETV6-NTRK3 fusion gene in, 136 imaging appearance, 21–37. See also magnetic extrarenal rhabdoid tumor, 920–921 genetics, 302 resonance imaging (MRI); fibroblastic tumors, 48 pathology, 302 radiologic evaluation of soft tissue fibromyxoid sarcoma, low-grade, 360 infantile hemangioendotheliomas. See juvenile tumors gastrointestinal stromal tumor, 72, 76, 80, capillary hemangioma angiomatous lesions, 25–30 81, 501 infantile melanotic neuroectodermal tumor, calciphylaxis, 879 glial fibrillary acidic protein, 80 1033 chondroma of soft parts, 862–863 granular cell tumor, 700–703 infantile myofibroma, 342, 1024 cystic masses, 34–36 hemangiopericytoma, 52–53, 77–79, infantile myofibromatosis (IM), 1024 elastofibroma, 36 343–345 infection-associated (iatrogenic) lymphedema, extraskeletal mesenchymal histiocytic markers, 66–68 4 chondrosarcoma, 870–871 immunoblotting, 86 infiltrating ductal carcinoma, 136 extraskeletal osteosarcoma, 888 inflammatory myofibroblastic tumor, infiltrative glomus tumor, 653 fibrodysplasia ossificans progressiva, 884 300–301 inflammatory malignant fibrous histiocytoma fibro-osseous pseudotumor of the digits, Kaposi’s sarcoma, 641 (MFH), 380–383, 444 887 keratins (cytokeratins), 68–74 inflammatory myofibroblastic tumor (IMT), lesion specificity, 18–21 KIT, 80–81 110–111, 135, 204, 256, 297, 301, lipomatous lesions, 21–25 malignant mesothelioma, 822–825 477 myositis ossificans, 882–884 melanoma markers (non-S100 protein), in children, 504 neurogenic tumors, 30–34 63–66 clinical features, 297–298 pigmented villonodular synovitis, 36–37 Merkel cell carcinoma, 73, 838 differential diagnosis, 301 PMT, 872–873 metastatic melanoma, 731 gene expression studies, 300–301 synovial chondromatosis, 865 mixed tumor/myoepithelioma, 793–795 genetics, 301 tophaceous pseudogout, 878 MPNST, 707 immunohistochemistry, 300–301 tumoral calcinosis, 877 MRNA analysis, 86 pathology, 298–300 imatinib/imatinib mesylate (Gleevec/Glivec), muscle cell markers, 51–56 terminology, 297 136–137, 358, 504 myoepithelioma, 793–795 inflammatory myxohyaline tumor, of the distal clinical studies of, 247 myxoid liposarcoma, 451 extremities. See acral for DFSP, 358–359 neck paragangliomas, 769 myxoinflammatory fibroblastic for GIST, 81, 491, 1066, 1070–1071 neural/neuroendocrine specific markers, sarcoma (AMIFS) resistance to, 137, 503, 1066, 1073 56–59 inflammatory pseudotumor, 297 NCI-sponsored trial, 504 neurofibroma, 677 inflammatory (lymphocyte-rich) immunoblotting (Western blotting), 86 neurothekeoma, 289 well-differentiated liposarcoma, immunoglobulin heavy chain (IgH) osteocalcin, 81 436–438 rearrangement, 982, 985 protein/gene expression demonstration inguinal well-differentiated liposarcoma, immunohistochemistry (other methods), 86–87 435 alveolar soft part sarcoma, 934–935 psammomatous melanotic schwannoma, intensity-modulated radiation therapy anaplastic lymphoma kinase (ALK), 75 689 (IMRT), 1061 angiomatoid (malignant) fibrous rhabdomyosarcoma, 53, 59, 62, 72 interdigitating reticulum cell sarcoma, 733, histiocytoma, 293 filament proteins, 566–567 1008 angiomyolipoma, 536 myogenic regulatory proteins, 567–568 intermetatarsal compression neuritis. See angiosarcoma, 634–636 myoglobin, 567 Morton neuroma

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Index 1095

intraabdominal desmoid fibromatosis, 504 differential diagnosis, 633, 641–642 laryngeal sarcomatoid carcinoma, 839 intraabdominal desmoplastic small round cell endemic KS, 637 laryngeal well-differentiated liposarcoma, 435 tumor, 81 epidemic KS, 636–637 latent nuclear antigen-1 (LNA-1), 641 intraabdominal leiomyosarcoma, 477 etiology/pathogenesis, 636 Ledderhose disease. See palmar and plantar intraabdominal lipoma, 396, 398 iatrogenic immunosuppression-associated, fibromatosis intraabdominal liposarcoma, 448 637 leiomyoma, 52–53 intraabdominal lymphangioma, 606 immunohistochemistry, 47, 48, 50, 51, 76, in abdominal soft tissues, 477 intraabdominal neuroendocrine carcinoma, 641 of female external genitalia and uterus, 478 843 pathology, 637–641 immunohistochemistry, 54, 84 intraabdominal solitary fibrous tumors vs. aneurysmal fibrous histiocytoma, 229 of male external genitalia, 466 (SFTs), 335 karyotype descriptions, 107–108 of muscularis mucosae, 469 intraabdominal wall desmoids, 244–245 keloid, 204 leiomyomatosis, 467. See also intravenous intraarticular masses. See pigmented keratinizing squamous carcinomas, 73 leiomyomatosis villonodular synovitis; synovial keratins (cytokeratins), 68–74 leiomyomatosis peritonealis disseminata. See chondromatosis background/overview, 69–71 peritoneal leiomyomatosis intralymphangial stromal myosis. See of basal cells of complex epithelia leiomyosarcoma, 2, 3, 52, 1038–1039 endometrial stromal sarcoma K5, 73 CDKN2A in, 141 intramural leiomyoma, 467 K14, 73 cutaneous leiomyosarcoma, 474 intramuscular hemangioma, 585–586 K17, 73 differential diagnosis, 297, 301, 504, 1039, intramuscular myxoma (IMM), 308–309, of corneal epithelium of eye (K3/K12), 74 1047, 1078 1044 of epithelia of biphasic synovial sarcoma EBV-associated leiomyosarcoma, 485–486 clinical features, 308–309 (K6/K16), 74 EBV-related, 460, 485–486 differential diagnosis, 309, 451, 518, high molecular weight (K2, K9, K11), 74 gastrointestinal leiomyosarcoma, 475–476 1021–1022, 1028 history/terminology, 68–69 genetics, 474 genetics, 309 of internal squamous epithelia/urothelium HRAS/KRAS mutations, 128 pathology, 309 (K4/K13), 73 immunohistochemistry, 52–53, 54, 58, 63, intramuscular nodular fasciitis, 185 of keratinizing stratified epithelia 67, 72, 75, 473–474 intramuscular well-differentiated liposarcoma, K1, 73 intraabdominal leiomyosarcoma, 477 434, 435 K10, 73–74 lung leiomyosarcoma, 476 intraneural neurofibroma, 669–672 of simple, nonstratified epithelia oral tract leiomyosarcoma, 476 intraosseous enchondroma, 862 K7, 72–73 organ-based, 476 intrathoracic lymphangioma, 606 K8, 71–72 paratesticular leiomyosarcoma, 476 intrathoracic synovial sarcoma, 787 K18, 71–72 pathology of, 470–473 intravascular B-cell lymphoma, 989 K19, 72 penile leiomyosarcoma, 476 intravascular fasciitis, 182, 189 K20, 71–72 pleomorphic leiomyosarcoma, 380 intravascular pyogenic granuloma, 579 of skin adnexa (K15), 74 prostate leiomyosarcoma, 476 intravenous leiomyomatosis, 480–481 Ki-67 monoclonal antibody, 82–83 retroperitoneal leiomyosarcoma, 444, irritation fibroma of oral cavity, 219–220 Kiel Pediatric Tumor Series, 898 475 ischemic fasciitis (atypical decubital Kimura disease, 592, 593 sinonasal tract leiomyosarcoma, 476 fibroplasia), 196–197 KIT, 80, 81, 492, 501, 502 soft tissue/visceral leiomyosarcoma Italian Sarcoma Study Group, 1074 KIT/PDGFRA, mutational activation of, (excluding uterine), 470–477 136–137 subcutaneous and intramuscular jaws (odontogenic), myxoma of, 325 KIT tyrosine kinase inhibitor drug therapy, leiomyosarcoma, 474–475 JAZF1-JJAZ1 fusion gene transcripts, 146 491, 504. See also imatinib/imatinib ultrastructure, 474 jugulotympanic paraganglioma, 770 mesylate urinary bladder leiomyosarcoma, 476 juvenile capillary hemangioma, 575–578 Klippel-Trenaunay syndrome, 603, 628 uterine leiomyosarcoma, 84, 483–485 clinical features, 576 Knudson, Alfred, 127, 139–140, 143, 144–145. of vena cava (and other veins), 476 pathology, 576–578 See also “two-hit hypothesis” lesion specificity by imaging, 18–21 related terms/entities, 578 Knudson-Strong “two-hit” hypothesis, 897 intrinsic characteristics, 20–21 juvenile fibromatosis, 238, 267, 885 KRAS codon mutations, 128 location, 19–20 juvenile hyaline fibromatosis, 279–280 leukemia, 3, 68 juvenile variant of DFSP. See giant cell labia majora LHFP (lipoma HMGA2/HMGIC fusion fibroblastoma pedunculated fibroepithelial skin polyps, partner) gene, 397 juvenile xanthogranuloma, 66, 67, 229, 510 Li-Fraumeni syndrome (LFS), 141, 896 1002–1004 vulvar angiomyofibroblastoma, 54, 80, limb perfusion/hyperthermia for non-GIST juxtaarticular myxoma, 309–312 478, 512 sarcomas, 1075 Langerhans cell histiocytosis, 67, 1000–1002 lipidized fibrous histiocytoma, 230 Kaposiform hemangioendothelioma, 578, Langerin, 1000–1002 lipid-rich rhabdomyosarcoma, 564 589–590 large B-cell lymphoma, diffuse, 988 lipoblastoma, 23, 420–423, 447, 451, Kaposi-like infantile hemangioendothelioma, large cell anaplastic lymphoma, 72, 75, 380 1018–1019 589–590 large cell carcinoma, 842 lipoblastomatosis, 23 Kaposi’s sarcoma (KS), 3, 44, 222, 402–403, large cell “proximal type” epithelioid sarcoma, lipodystrophy (fat atrophy), 423 617. See also human herpes virus 8 792 lipofibromatosis, 265–268, 290, 421 classic (chronic) KS, 636 laryngeal inflammatory myofibroblastic lipogranuloma, 423, 441 described, 636 tumors (IMTs), 298 lipoleiomyoma, 477

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1096 INDEX

lipoma arborescens (synovial lipomatosis), lymphangioma, 25, 29, 50, 64, 73, 450, vs. reactive mesothelial proliferation, 19–20, 397–398 605–607 825–826 lipoma-like liposarcoma, 436 clinical features, 606 vs. vascular tumors, 829–830 lipomas, 1017–1018 differential diagnosis, 606–607, 813 gross features, 818 angiolipoma, 402–403 pathology, 606 histopathology, 818 chondroid lipoma, 414–418 lymphangiomatosis, 611–612 immunohistochemistry, 822–825 differential diagnosis, 396, 448, 1017 lymphangiomyoma/lymphangiomyomatosis, multicystic peritoneal mesothelioma, 811, fat atrophy (lipodystrophy), 423 63, 536–537 813 fat necrosis/lipogranuloma, 423 lymphangiosarcoma, 617 ultrastructure, 825 hemosiderotic fibrohistiocytic lipomatous lymphatic vascular lesions variants lesion/tumor, 420 benign lymphangioendothelioma, 609–611 biphasic mesothelioma, 819–822 HMGA2 in, 137 lymphangioma, 605–607 desmoplastic mesothelioma, 822 lipoma arborescens (synovial lipomatosis), lymphangiomatosis, 611–612 peritoneal mesothelioma, 818 397–398 lymphedema-associated angiosarcoma, pleural mesothelioma, 818 lipomatoses, 401–402 626–628 sarcomatoid mesothelioma, 822 lumbosacral/spinal cord associated, 398 lymphedema in the obese, 426–427 solid epithelial mesothelioma, 819 with metaplastic cartilage and bone, 397 lymph node metastases of melanomas, 293 tubulopapillary malignant myelolipoma, 403–405 lymphoblastic lymphoma, 904 mesothelioma, 818–819 myolipoma, 418–420 lymphoid hyperplasias well-differentiated papillary mesothelioma, nevus lipomatosus superficialis, 397 follicular lymphoid hyperplasia, 982 813–816 paraffinoma/sclerosing lipogranuloma, hyaline vascular Castleman disease, WT1 overexpression in, 143 423–425 982–984 malignant mixed mesodermal tumor. See pleomorphic lipoma, 405–409 Kimura disease, 984–986 carcinosarcoma sclerotic lipoma, 409–413 lymphoma, 1, 379, 559 malignant mixed mullerian¨ tumor (MMMT), soft tissue lipomas, 393–397 differential diagnosis, 520 1078. See also carcinosarcoma spindle cell lipoma, 405–409 immunohistochemistry vs. electron malignant perineurioma, 699 thymolipoma, 399 microscopy, 9 malignant peripheral nerve sheath tumor visceral lipoma, 398–399 p53 mutation in, 141 (MPNST), 3, 13, 20, 30, 32–33, 73, lipomatoses, 401–402 Lyon’s hypothesis, 148 80, 631, 1035 lipomatosis of the nerve, 399–401 lysozyme (muramidase) bacteriolytic enzyme, clinical features, 706 lipomatous angioleiomyoma, 466 66–67 described, 704–706 lipomatous hemangiopericytoma (HPC), differential diagnosis, 711, 1026, 1035, 343 Maffucci syndrome, 628, 862 1047, 1049 lipomatous hypertrophy of the interarterial magnetic resonance angiography (MRA), 13 with divergent differentiation, 711–712 septum, 399 magnetic resonance imaging (MRI), 11, 13–15 epithelioid MPNST, 712–714 lipomatous lesions, 21–25 of hemangiomas, 26–27 from ganglioneuroma and liposarcomas, 36 of neurogenic tumors, 30–33 pheochromocytoma, 714 dedifferentiated liposarcoma, 24–25, malakoplakia, 970 genetics, 711 441–445 male angiomyofibroblastoma-like tumor, immunohistochemistry, 61, 62, 63, 72, differential diagnosis, 342, 345 514–515 707 HRAS/KRAS mutations, 128 male external genitalia, leiomyoma, 466 malignant glandular MPNST, 712 intraabdominal liposarcoma, 448 malignant fibroblastic tumors. See pathology, 706–707 lipoma-like liposarcoma, 436 fibrosarcoma; malignant fibrous treatment of, 1064 myxoid liposarcoma, 447–453 histiocytoma Triton tumor, 712 pleomorphic liposarcoma, 453–456 malignant fibrous histiocytoma (MFH), 3, 11, ultrastructure, 711 round cell liposarcoma, 110, 447–450, 453 36, 52, 67, 77. See also giant cell vs. BPNST, 33–34 spindle cell liposarcoma, 438 malignant fibrous histiocytoma; malignant tenosynovial giant cell tumor, treatments, 432 inflammatory malignant fibrous 940–961 well-differentiated liposarcoma, 23–24 histiocytoma; pleomorphic malignant Triton tumor, 712 liquified hematomas, 35–36 malignant fibrous histiocytoma mammary-type myofibroblastoma (MTMF), littoral cell angioma, 629 HRAS/KRAS mutations, 128 1024 lobular capillary hemangioma, 578–579 immunohistochemistry, 72 massive localized lymphedema in the obese, lobular mammary carcinomas, 80, 520 malignant glomus tumor, 650–653 426–427 localized interdigital neuritis. See Morton malignant granular cell tumor, 703 Masson tumor, 600–601 neuroma malignant melanoma, 51, 58, 81, 737–739 mast cell tryptase, 997 low-grade fibromyxoid sarcoma, 453, 1028 malignant mesenchymoma, 1, 444 mast cell tumors mastocytoma, 80 low-grade myxofibrosarcoma, 314, 364, 1028 malignant mesothelioma, 79. See also mastocytoma, 80, 996–997 low-grade sarcoma, 669 mesothelioma mastocytosis, 996–997 lumbosacral lipoma, 398 adenomatoid tumor, 811–813 maxillofacial lipoma, 405 lung adenocarcinomas, 72 differential diagnosis Mazabraud syndrome (fibrous dysplasia), 20, lung leiomyosarcoma, 476 vs. other carcinoma, 827–829 309 Lyme disease synovitis, 940–941 vs. primary mesenchymal tumors, 829 McCune-Albright syndrome, 309 lymphadenopathic Kaposi’s sarcoma, 637 vs. primary peritoneal/metastatic MDM2 (murine double minute) gene and lymphangioendothelioma, 631 serous papillary carcinoma, 827 protein, 83–84, 139, 441

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Index 1097

media for specimen transport, 105–106 desmoplastic mesothelioma, 822 CDKN2A gene, 141–142 mediastinal lipoma, 398 etiologic factors, 818 classical oncogenes/tumor suppressor mediastinal tumors, 493 incidence of, 816 genes, 127 mediastinal well-differentiated liposarcoma, multicystic peritoneal mesothelioma, 813 clonality assessment using HUMARA, 434, 435 peritoneal mesothelioma, 818 148–149 medullary thyroid carcinoma, 662, 854 sarcomatoid mesothelioma, 822 COLIA1-PDGFB fusion gene, 137 medulloblastomas, 57, 59, 142 solid epithelial mesothelioma, 819 detection of minimal residual disease, melan-A (MART-1, melanoma antigen tubulopapillary malignant mesothelioma, 147–148 recognized by T-lymphocytes), 64 818–819 epimutation, 127 melanocytes (and their tumors), 60 well-differentiated papillary mesothelioma, ETV6-NTRK3 fusion gene, 136 melanocytic neuroectodermal tumor of 813–816 EWSR1 involvement in fusion genes, infancy, 740–741 metal fragment deposition, synovia and 129–132 melanoma tendon pathology, 950 fusion genes/transcripts differential diagnosis, 379, 1030–1031 metaplastic bone, 887 detection, 145–146 immunohistory vs. electron microscopy, 9 metastatic adenocarcinoma, 1048 tumor-specificity determination, 146 lymph node metastases of, 293 metastatic adrenal cortical carcinoma, 852 inherited cancer syndromes, 145 melanoma markers (other than S100 protein), metastatic calcification, 879 mutational activation of KIT/PDGFRA, 63–66 metastatic carcinoma, 1, 202 136–137 CD63 (NK 1C3), 66 of breast, 841 neurofibromatosis type 1 gene (NF1), HMB-45 monoclonal antibody, 63 differential diagnosis, 454, 520, 1042 143–144 melan-A, 64 immunohistochemistry, 75 neurofibromatosis type 2 gene (NF2), microphthalmia transcription factor, 66 metastatic clear cell tumors, 739 144–145 tyrosinase, 64 metastatic ductal carcinoma, 841 oncogene amplification, 138–139 melanotic neuroectodermal tumor of infancy, metastatic epithelial tumors, 1, 59 oncogenic activation of receptor tyrosine 1033 metastatic gastrointestinal adenocarcinoma, kinases, 134 melphalan, 1075 846 P53, altered expression, 140–141 MEN2B syndrome, 664 metastatic lobular carcinoma, 841 pathologic fusion genes, 128–129 meningeal hemangiopericytoma, 343, 345 metastatic malignant melanoma, 1042, 1049 PAX-FKHR fusion genes, 132–133 meningeal solitary fibrous tumors (SFTs), metastatic melanoma, 1, 59, 67, 504, 726–733 RAS genes/RAS signaling pathways, 128 336–337 clinical features, 726–727 rearrangement of HMGA transcription meningioma, 77–79 differential diagnosis, 731–733, 1048 factor genes, 137–138 outside the central nervous system, genetics, 733 retinoblastoma gene (RB1), 139–140 744–750 immunohistochemistry, 731 selected techniques, 149–153 ectopic meningioma in paranasal soft pathology, 727 array CGH, 153 tissue, 746–748 metastatic meningioma, 749–750 cDNA microarrays, 153 metastatic meningioma, 749–750 metastatic neuroendocrine carcinoma, 56 comparative genomic hybridization, pulmonary meningioma nodules, 749 metastatic prostatic carcinoma, 851–852 153 scalp, 745–746 metastatic pulmonary adenocarcinoma, DNA/RNA extraction, 149–150 sinonasal, external ear, orbital 842 PCR amplification, 150–153 meningiomas, 748–749 metastatic pulmonary small cell carcinoma quantitative-PCR, 152 meningothelial hamartoma, 745–746 (SCC), 842–843. See also rapid amplification of cDNA ends, 151 meningothelial nodule, 745–746 neuroendocrine carcinoma RT-PCR, 151 meniscal cysts, 34 metastatic renal cell carcinoma, 847–849 sample preparation, 149 Merkel cell carcinoma, 57, 58, 72, 834–838 metastatic spindle cell carcinoma, 1047 SMARCB1 inactivation, 142 clinical features, 834–835 Mibelli angiokeratoma, 581 SS18-SSX fusion genes, 133–134 differential diagnosis, 838, 842 microphthalmia transcription factor (MITF), Wilms’ tumor gene (WT1), 142–143 genetics, 838 66 monomorphous round cell immunohistochemistry, 73, 838 microRNA gene family, 140 rhabdomyosarcoma, 559 pathology, 835–836 microscopy/automated image analysis systems, monophasic spindle cell synovial sarcoma, Merkel cell polyomavirus (MCV), 836–838 107 341, 780–783, 1026 mesenchymal chondrosarcoma, 342, 345 microvenular hemangioma, 598 monophasic synovial sarcoma, 72, 73, 787 mesenchymal tumors, 61–62, 81, 105, 106 Milroy disease, 627 Morton neuroma, 30, 32, 661–662, 664 mesenteric desmoids, 244 mixed tumor/myoepithelioma (MT/ME), mTOR inhibitors, 504. See also everolimus; mesenteric gastrointestinal tumor (GIST), 792–795, 1044 sirolimus 500 clinical features, 792–793 mucinous cystadenocarcinoma, 631 mesna with doxorubicin, ifosfamide, differential diagnosis, 795, 1044, 1051 mucoepidermoid carcinoma of salivary glands, dacarbazine (MAID), 1077 genetics, 795 131 mesodermal stromal polyp immunohistochemistry, 793–795 mucosa-associated lymphoid tissue (MALT) (pseudosarcomatous stromal pathology, 793 lymphoma. See extranodal marginal polyp), 508–510 molecular genetics of soft tissue tumors zone lymphoma mesothelioma, 50, 202. See also malignant ALK gene arrangements, 134–136 mucosal ganglioneuroma, 664 mesothelioma APC inactivation/β-catenin activation in mucosal melanomas, 64, 510 and asbestos, 817–818 Wnt pathway, 143 mucosal neuroma/ganglioneuromatosis, biphasic mesothelioma, 819–822 ASPL-TFE3 gene fusion, 134 662–664

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1098 INDEX

multicentric reticulohistiocytosis, 1005–1006 myxofibrosarcoma (MFS, myxoid malignant immunohistochemistry, 769 multicolor FISH (M-FISH), 108, 113 fibrous histiocytoma), 370–373, pathology, 768–769 multicystic peritoneal mesothelioma 1026–1028 vagal body paraganglioma, 768 (multilocular peritoneal inclusion clinical features, 370–371 necrobiotic granuloma, 966–967 cyst), 811, 813 differential diagnosis, 372, 444, 451, necrotizing fasciitis, 199–200 multiple desmoids, 246–247 1021–1022 clinical features, 200 multiple extra-adrenal myelolipoma, 405 genetics, 372–373 pathology, 200 multiple hamartoma syndrome, 215 pathology, 372 nerve growth factor receptor (low affinity), muscle cell markers, 51–56 surgical management, 1063 p75, 62 actins, 51–52 myxoid components in WDLS, 438 nerve sheath cellular alpha smooth muscle actin (SMA), myxoid fibrous histiocytoma (MFH), 61, 309 components/tumorigenesis 52–53 myxoid leiomyoma, 518 significance, 660–661 calponin, 54 myxoid liposarcoma (MLS), 15, 61, 110, nerve sheath myxoma, 80, 285, 289, 691 desmin, 53–54 447–453, 1021–1022 clinical features, 692 heavy caldesmon (HCD), 54 clinical features, 447–448 differential diagnosis, 694 HHF-35 (muscle-specific actin), 52 differential diagnosis, 309, 414, 421, 451, pathology, 692–694 myogenic regulatory factors/myogenin, 1021–1022 nerve sheath tumor-associated angiosarcoma, 54–56 EWSR1-DDIT3 (EWS-CHOP) fusion, 631 myoglobin, 56 132 nerve sheath tumors, 3, 13, 62, 514. See also sarcomeric actin, 53 EWSR1 involvement in, 129 malignant peripheral nerve sheath smooth muscle myosin, 53 genetics, 451–453 tumor; neurofibroma; muscular dystrophy, 396 histologic features, 448–451 perineurioma; schwannoma Musculoskeletal Tumor Society Staging HMGA2 in, 137 epithelioid peripheral nerve sheath tumor, System. See Enneking staging immunohistochemistry, 451 691 system pathology, 448 gingival granular cell tumor of newborn, MYC gene, 138 myxoid malignant fibrous histiocytoma 703–704 MYCN amplification, 138–139 (myxofibrosarcoma), 370–373, 451, granular cell tumor, 699–703 mycobacterial pseudotumor, 968–970 456 malignant granular cell tumor, 703 myeloid and related proliferations. See also myxoid MFH, 61 Morton neuroma, 30, 32, 661–662, 664 acute myeloid leukemia (AML) myxoid smooth muscle tumors of uterus, mucosal neuroma/ganglioneuromatosis, extramedullary myeloid tumor, 66, 80, 479–480 662–664 994–996 myxolipoma (ML), 1018 myxoma, 80, 285, 289, 691 mastocytoma/mastocytosis, 996–997 myxomas, 36, 256 neurofibromatosis 1, 669 myeloid leukemia, 80, 138 cardiac myxoma, 323–325 neuromuscular hamartoma, 661 myelolipoma, 403–405 cutaneous myxoid cyst, 314 Pacinian neuroma, 664 myeloperoxidase, 989, 996 intramuscular myxoma, 308–309 palisaded encapsulated neuroma, 664–666 myeloproliferative disorders, 441 juxtaarticular myxoma, 309–312 traumatic neuroma, 30, 32, 662 myoepithelial carcinoma (malignant mixed odontogenic (jaws) myxoma, 325 network-forming collagens, 182 tumor), 733, 796 ossifying fibromyxoid tumor, 325–331 neural fibrolipoma, 399–401 myoepithelioma, 80, 131, 792–795 superficial acral fibromyxoma (SAF), neural and neuroendocrine specific markers, myofibroblastic sarcoma (myofibrosarcoma), 321–323 56–59 188, 309, 314, 364–365 superficial angiomyxoma (SAM), 314–320 alpha-internexin, 59 myofibroblastoma, 224. See also cervicovaginal myxomatous neoplasms, 34, 36. See also chromogranin A, 58 myofibroblastoma extraskeletal chondrosarcoma; NB84 (neuroblastoma marker), 59 of the breast, 222–224 liposarcomas; malignant fibrous neurofilament (NF) proteins, 58–59 myofibroblasts, 181–182 histiocytoma; myxomas; neurogenic neuron-specific enolase (NSE), 58 myofibroma and myofibromatosis, 273–279 tumors synaptophysin, 56–58 clinical features, 273–275 myxopapillary ependymoma of soft tissue neurilemmoma (schwannoma), 30, 32–33 differential diagnosis, 279 (MPEST), 742, 1033–1035 neuroblastoma, 56, 58, 836, 906–915 pathology, 276–279 myxopapillary tumors, 80 ALK gene fusion in, 135–136 myofibrosarcoma (myofibroblastic sarcoma), ancillary diagnostic techniques, 911–912 188, 309, 314, 364–365 nasal glioma, 741–742 clinical features, 907–908 myogenic regulatory factors (MyoD1, Myf3 nasal-type natural killer cell/T-cell differential diagnosis, 914–915 antibody), 54–56 (NK/T)-cell lymphoma, 992 genetics, 912–914 myogenic sarcoma, 379 nasopharyngeal angiofibroma, 269–271 gross/microscopic features, 908–911 myogenin (Myf4 antibody), 54–56 clinical features, 269–270 history, 907 myolipoma, 418–420 differential diagnosis, 271 prognosis/clinical outcome, 914 myopericytic tumors, 342 pathology, 270–271 neuroblastoma-like schwannoma, 684 myopericytoma, 658 National Cancer Institute of Canada, 1074 neuroectodermal tumors, 81. See also myositis ossificans (heterotopic bone natural killer cell/T-cell (NK/T)-cell meningioma, outside the central formation), 11, 881–884, lymphoma, 992 nervous system 1042–1044 NB84 (neuroblastoma marker), 59 cellular blue nevus, 724–726 clinical features, 881–882 neck paragangliomas, 767–770 clear cell sarcoma of tendons and differential diagnosis, 1042 carotid body paraganglioma, 768 aponeuroses, 736–739 imaging/pathology, 882–884 differential diagnosis, 769–770 desmoplastic melanoma, 733–735

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Index 1099

ectopic glial tissue (nasal glioma/soft tissue desmin in, 290 oncogenes (classical) gliomatosis), 741–742 differential diagnosis, 289 amplification, 138–139 ependymoma fibrohistiocytic tumor neurothekeoma, 66 and tumor suppression genes, 127 abdominal, 744 immunohistochemistry, 289 oncogenic viruses, 3 myxopapillary, 742 nevus lipomatosus superficialis, 397 oncology of soft tissues tumors gastrointestinal clear cell sarcoma, NF1 gene, 128, 143, 144, 660, 669, 678–679 background information, 1070 739–740 NF2 gene, 144, 145, 679, 685, 686, 691, 696 future directions, 1079–1080 melanocytic, of infancy, 740–741 NMP-ALK fusion protein, 135 gastrointestinal stromal tumors, meningioma outside the CNS, 744–750 nodal lymphangiomyoma, 536 1070–1073 metastatic melanoma, 1, 59, 67, 504, nodular fasciitis, 21, 52, 54, 76, 182–189, 1022 adjuvant therapy for, 1072 726–733 clinical features, 182–184 dasatinib for, 1073 neuroendocrine carcinoma, 56, 58, 77–79, differential diagnosis, 188–189, 251, imatinib, 81, 491, 1066, 1070–1071 842–843. See also metastatic 255–256, 1022, 1024, 1030 palliative surgery for, 1072 pulmonary small cell carcinoma genetics, 189 sunitinib/sunitinib malate for, 504, neurofibroma (NF), 30, 32–33, 48, 62, 669, pathology, 184–185 1066, 1071 1033 nodular hidradenoma (NH), 1048 tyrosine-kinase inhibitor resistant, cellular neurofibroma, 669 nodular tenosynovitis, 951–952 1072–1073 cutaneous neurofibroma, 669, 672, non–ACTH-dependent Cushing syndrome, non-GIST sarcomas, 1073–1079 675 320 adjuvant/neoadjuvant chemotherapy, differential diagnosis, 677–678 non–asbestosis related mesothelioma, 143 1073–1075 diffuse neurofibroma, 675 noncutaneous (systemic) anaplastic large cell advanced disease/combination immunohistochemistry/ultrastructure, lymphoma (ALCL), 992–994 chemotherapy, 1077 677 non-involuting congenital hemangioma, 575, advanced disease/systemic therapy, intraneural neurofibroma, 669–672 578 1076–1077 pigmented neurofibroma, 675 non-mesenchymal skin tumors, 1 angiosarcoma, 1078 plexiform neurofibroma, 672–675, non-neoplastic lesions chemoradiotherapy, 1075 679 amyloid tumor, 976–978 desmoid tumor, 1079 transformation of, 677 cotton-fiber granuloma, 973–974 intensification of treatment, 1077 neurofibromatosis, 896 deep granuloma annulare, 966–967 limb perfusion/hyperthermia, 1075 neurofibromatosis 1 (NF1, von epidermal inclusion cyst rupture, management of recurrent tumors, Recklinghausen’s disease), 20, 128, histiocytic reaction, 972–973 1075–1076 143–144, 551, 669 gadolinium-associated fibrosis, 976 pediatric sarcoma in adults, 1078–1079 described, 678 malakoplakia, 970 response by site/histology, 1077–1078 gene alterations, 678–679 mycobacterial pseudotumor, 968–970 uterine sarcoma, 1078 subgroups, 679 organizing hematoma, 965–966 open biopsy (evaluation method), 7 neurofibromatosis 1 (NF1) patients, 501 polyvinylpyrrolidine (PVP) accumulation, oral/maxillofacial lipoma, 405 neurofibromatosis 2 gene (NF2), 144–145, 973 oral solitary fibrous tumors (SFTs), 336 685–686 pulse granuloma, 967–968 oral tract leiomyosarcoma, 476 neurofibromatosis 2 syndrome, 685–686 thorium oxide deposition (thorotrast), orbital meningioma, 748–749 diagnosis, 685–686 974–976 orbital solitary fibrous tumors (SFTs), pathogenesis, 686 xanthogranulomatous inflammation, 335–336 neurofilament (NF) proteins, 58–59 970–971 organizing hematoma, 965–966 neurogenic tumors, 21, 36. See also malignant xanthoma, 971–972 Oroya fever, 602–603 peripheral nerve sheath tumor; non-osseous sarcomas, 1 ossifying fasciitis, 182 Morton neuroma; neurilemmoma; nonrhabdomyosarcomatous soft tissue ossifying fibromyxoid tumor, 61, 325–331 neurofibroma; traumatic neuroma sarcoma, 4 osteoarthritis, 942 imaging appearances, 30–34 Noonan syndrome, 128 osteogenic sarcoma, 1078 neuroma. See Morton neuroma; mucosal NR4A3 gene, 131, 870 osteolipoma, 397 neuroma/ganglioneuromatosis; NTRK3 gene, 136, 302 osteosarcoma, 3, 11 palisaded encapsulated neuroma nuchal fibrocartilaginous pseudotumor, 211 ovarian adenocarcinomas, 72, 79 (solitary circumscribed neuroma); clinical features, 211 ovarian carcinoma, 62, 81, 852–854 traumatic neuroma (amputation pathology, 211 ovarian Leydig cell tumors, 64 neuroma) nuchal-type fibromas, 208–211 neuromuscular choristoma, 549, 661 clinical features, 208–210 p27, 84, 451 neuromuscular hamartoma, 661 differential diagnosis, 269 p53 (TP53) cell cycle regulator, 83 neuron-specific enolase (NSE), 58 pathology, 210–211 altered expression of, 140–141 neurothekeoma, 228–229 nuclear medicine, 13 Pacinian neuroma (Pacinian neurofibroma), in children, 285–289 Nutlin-3a treatment for liposarcoma, 664 benign fibroblastic/myofibroblastic 432 palisaded encapsulated neuroma (solitary proliferations, 285–289 circumscribed neuroma), clinical features, 285–286 ochronosis, alkaptonuric, 950–951 664–666 differential diagnosis, 289, 290 odontogenic (jaws) myxoma, 325 clinical features, 664–666 immunohistochemistry, 289 olfactory neuroblastoma, 750 pathology, 666 pathology, 286 Ollier disease, 862 palisaded myofibroblastoma, 220–222 clinical features, 285–286 omental gastrointestinal tumor (GIST), 500 palisaded-vacuolated GIST, 494

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1100 INDEX

palmar and plantar fibromatosis, 238–243 PEComas. See perivascular epithelioid cell pigmented dermatofibrosarcoma protuberans clinical features, 238 tumors (PEComas) (DFSP), 354 differential diagnosis, 240 pedal (foot) hemangioma, 585 pigmented neurofibroma, 63 genetics, 240–243 pediatric angiosarcoma, 631 pigmented villonodular synovitis (PVNS), 21, pathology, 239–240 pediatric gastrointestinal stromal tumor 36–37, 951–952 treatment, 239 (GIST), 500–501 pilar leiomyoma (piloleiomyoma), 463 pancreatic adenocarcinoma, 846 Pediatric Oncology grading system, 4 piloleiomyoma. See pilar leiomyoma panniculitis-like T-cell lymphoma (PTCL), pedunculated fibroepithelial skin polyps of the (piloleiomyoma) 991–992 skin of the labia majora, 510 pituitary adenomas, 137, 320 panniculitis ossificans, 182 pelvic desmoids, 244–245 planar xanthoma (xanthelasma), 971–972 papillary endothelial hyperplasia (Masson pelvic lipomatosis, 401 plasma cell granuloma, 297 tumor), 600–601 pelvic tumors, 906 plasmacytoma/plasmablastic B-cell papillary intralymphatic angioendothelioma penile fibromatosis, 243 lymphoma, 989–991 (Dabska tumor), 617–619 penile leiomyosarcoma, 476 plastic induration of the penis. See penile papillary mesothelial hyperplasia, 825 penile myointimoma, 189 fibromatosis parachordoma, 795–796 pericytic (perivascular) tumors, 1042 platelet-derived growth factor receptor alpha paraffinoma/sclerosing lipogranuloma, perilabral cysts, 34 (PDGFRA) gene, 136–137 423–425 perilobular hemangioma, 628 pleomorphic adenomas of salivary gland, 137 paraganglioma, 56, 57, 58, 59, 61, 67, 496, perineurioma, 75, 694–699 pleomorphic fibroma of the skin, 215–216 501, 750 differential diagnosis, 697–699 pleomorphic hyalinizing angiectatic tumor cauda equina/spinal canal paraganglioma, intraneural perineurioma, 695 (PHAT), 384–387 773 malignant perineurioma, 699 pleomorphic leiomyosarcoma, 380 differential diagnosis, 935 other soft tissue tumors, 696–697 pleomorphic lipoma, 405–409, 1019 duodenal gangliocytic paraganglioma, retiform (reticular) perineurioma, 696 clinical features, 405–407 770–773 sclerosing perineurioma, 695–696 differential diagnosis, 409, 441, 1019 jugulotympanic paraganglioma, 770 pariosteal fasciitis, 182 genetics, 409 overview, 755–756 peripheral neuroepithelioma, 129 pathology, 407–408 pheochromocytoma of the adrenal, peripheral primitive neuroectodermal tumor pleomorphic liposarcoma, 453–456 758–761 (pPNET), 110, 897, 901, 918 pleomorphic malignant fibrous histiocytoma pheochromocytoma of the urinary peripheral soft tissue inflammatory (MFH, pleomorphic bladder, 761–767 myofibroblastic tumors (IMTs), undifferentiated sarcoma), 373–379, retroperitoneal extra-adrenal 298 444 paraganglioma, 761 peritoneal leiomyomatosis, 79, 481–483 biology of, 376, 378 syndromes, 756–758 peritoneal mesothelioma, 818 clinical features, 376 Carney-Stratakis syndrome, 758 perivascular epithelioid cell tumors concept evolution, 373–376 familial paraganglioma (PGL) (PEComas), 63, 393, 531, 538 differential diagnosis, 378–379 syndromes, 757 abdominal tumor pathology, 539–542 electron microscopy, 378 multiple endocrine neoplasia 2A/2B, behavior of, 539 genetics, 378 756 genetics, 542 pathology, 376 neurofibromatosis 1, 20, 128, 143–144, renal capsular tumors, 538 pleomorphic rhabdomyosarcoma (PRMS), 551, 669, 757 soft tissue/cutaneous PEComas, 542 561–562, 1038 Von Hippel-Lindau disease, 756–757 uterine tumors, 538–539 pleural mesothelioma, 818 thoracic paraganglioma, 767 peroxisome proliferator-activated receptor pleural-thoracic solitary fibrous tumors paratesticular leiomyosarcoma, 476 gamma (PPAR gamma), 432 (SFTs), 335 PAS-positive Peyronie’s disease. See penile fibromatosis plexiform fibrohistiocytic tumor (PFHT), in biphasic synovial sarcoma, 780 phenoxyacetic-acid herbicides, 3 228–229, 289–292 in carcinosarcoma, 522 pheochromocytoma, 59, 662 clinical features, 289–290 in chondroid lipoma, 415 of the adrenal, 758–761 differential diagnosis, 290, 383 in psammomatous melanotic schwannoma, clinical features, 758 genetics, 292 688 genetic syndrome, 758–760 pathology, 290 in pulmonary clear cell “sugar” tumor, 538 immunohistochemistry, 760–761 plexiform neurofibroma, 672–675, 679 pathologic fusion genes, 128–129 malignancy prediction, 760 plexiform schwannoma, 683–684 PAX-FKHR fusion genes, 132–133 non–syndrome related genetics, 761 “plywood fibroma,” 214–215 PAX-FOX01A fusion, 133 pathology, 758 PMT-associated oncogenic osteomalacia, PAX-FOXO1A fusion gene, 146 ultrastructure, 761 875 PAX3-AFX fusion gene, 147 of the urinary bladder, 761–767 PMT. See phosphaturic mesenchymal tumor PAX3-FOXO1A fusion gene, 147 clinical features, 761–765 of mixed connective tissue variant PAX3-NCOA1 fusion gene, 147 pathology, 765–767 (PMT) PAX7-FOXO1A fusion gene, 147 phosphaturic mesenchymal tumor of mixed podoplanin (D 2-40), 50–51 PBX1 (pre–B-cell leukemia transcription connective tissue variant (PMT), POEMS syndrome, 586–587 factor 1), 131 871–875 polyclonal marrow plasmacytosis, 323 PCR-based assays, 146, 150–153 phyllodes tumor, 434 polyethylene wear particles, 947 PDGFRA mutations in gastric GISTs, 500, pigmented dermatofibrosarcoma (Bednar polyostotic fibrous dysplasia of bone, 309 502–503 tumors), 675 polyvinylpyrrolidine (PVP) accumulation, 973

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Index 1101

poorly differentiated embryonal pulmonary epithelioid renal cancer. See hereditary leiomyomatosis rhabdomyosarcoma, 302 hemangioendothelioma, 620 and renal cell cancer poorly differentiated myxoid (round cell) pulmonary glomus tumor, 649 renal tumors liposarcomas, 61 pulmonary inflammatory myofibroblastic adenocarcinoma, 67 poorly differentiated sinonasal carcinoma, 750 tumors (IMTs), 298 angiomyolipoma, 64, 542 poorly differentiated synovial sarcoma, 59, 72, pulmonary lymphangiomyoma, 536 ASPL-TFE3 gene fusion in, 134 73, 345, 783, 787 pulmonary lymphangiomyomatosis tumor, capsular tumors, 538 popliteal cysts, 35 531 carcinoma, 58, 62, 72, 81 positron emission tomography (PET), 13, 23. pulmonary meningioma nodules, 749 clear cell carcinoma, 454, 564 See also FDG PET pulmonary small cell carcinoma, 504, 836, hereditary leiomyomatosis and renal cell postinflammatory tumor, 297 838 cancer, 463 POU 5F1 (OCT3/4) sequence-specific pulse granuloma (giant cell hyaline metastatic renal cell carcinoma, 847–849 transcription factor, 131 angiopathy), 967–968 pelvic carcinoma, 650 precursor B-cell lymphoblastic lymphoma, sarcomatoid renal cell carcinoma, 849–851 988–989 quantitative-PCR (Q-PCR), 152 reporting (evaluation method), 7 primary cutaneous anaplastic large cell resection specimen (evaluation method), 7 lymphoma, 994 RACE (rapid amplification of cDNA ends), respiratory tract lipomas, 399 primary epithelial tumors, 1 151 reticulohistiocytoma (solitary epithelioid primary neuroendocrine carcinoma, 56, radiation-associated angiosarcoma, 626 histiocytoma), 66, 68, 289, 834–838 radiation-induced sarcomas, 3 1004–1005 primary peritoneal/metastatic serous papillary radiation treatment reticulohistiocytosis, 67 carcinoma, 827 complications, timing of, 1061 retiform hemangioendothelioma, 619 primitive leukemias, 49 external beam radiation therapy, 1061 retinoblastoma gene (RB1), 139–140 primitive neuroectodermal tumor (PNET), extremity/truncal radiation, 1061 retinoblastoma gene product (RB), 84 30, 57, 58, 142, 1048–1049. See also intensity modulated radiation therapy, retroperitoneal angiomyolipoma (AML), 531, Ewing sarcoma 1061 536 proliferative fasciitis, 189–195 in retroperitoneal sarcoma, 1065 retroperitoneal dedifferentiated liposarcoma proliferative myositis, 195–196 radiologic evaluation of soft tissue tumors (DDLS), 441, 444 prostate cancer, ETS gene family angiography, 13 retroperitoneal extra-adrenal paraganglioma, involvement, 129 computed tomography (CT), 11, 13–15 761 prostate leiomyosarcoma, 476 goals of, 11 retroperitoneal fibrosis, 200–204 prostatic carcinoma, 72, 851–852 lesion specificity by imaging, 18–21 retroperitoneal leiomyoma, 477 psammomatous melanotic schwannoma, 63, magnetic resonance imaging (MRI), 11, retroperitoneal leiomyosarcoma, 444, 475 320, 687–691 13–15 retroperitoneal lymphangiomyoma, 531 clinical features, 688 nonspecific imaging appearances, 37 retroperitoneal pleomorphic sarcomas, 441 differential diagnosis, 689–691, 731 nuclear medicine, 13 retroperitoneal sarcoma, surgical immunohistochemistry, 689 radiographs, 11 management, 1065–1066 pathology, 688–689 specific imaging appearances, 21–37 locally recurrent retroperitoneal sarcoma, pseudomalignant heterotopic ossification, angiomatous lesions, 25–30 1066 881–884 “cystic” masses, 34–36 radiation treatment, 1065 pseudomalignant osseous tumor of soft tissue, lipomatous lesions, 21–25 subtypes/goals of surgery, 1065 881–884 neurogenic tumors, 30–34 retroperitoneal well-differentiated pseudorheumatoid nodule, 966–967 staging, 17–18 liposarcoma, 434–435 pseudosarcomatous fasciitis. See nodular ultrasound, 15–17 reverse transcriptase-polymerase chain fasciitis rapid amplification of cDNA ends (RACE), reaction (RT-PCR) analyses, 105, pseudosarcomatous fibromyxoid tumor, 297 151 108, 116, 151 pseudosarcomatous fibrous histiocytoma, rapidly involuting congenital hemangioma, rhabdomyoma, 546, 1035–1036 232 575, 578 adult rhabdomyoma, 548–549 pseudosarcomatous myofibroblastic spindle RAS genes/signaling pathways, 128, 136. See differential diagnosis, 906, 1021 cells, 182 also KRAS codon mutations fetal rhabdomyoma, 546–548 pseudosarcomatous myofibroblastic tumor, reactive angioendotheliomatosis, 603 genital (vaginal) rhabdomyoma, 549 297 reactive epidermal hyperplasia, 603 with rhabdoid features, 565 pseudosarcomatous polyp, 510 reactive mesothelial proliferation, 825–826 rhabdomyoma-like rhabdomyosarcoma, pseudosarcomatous stromal polyp reactive vascular proliferations, 601 564–565 (mesodermal stromal polyp), real-time PCR, 152 rhabdomyomatous mesenchymal hamartoma, 508–510 receptor tyrosine kinases, oncogenic activation 549–550 pseudotumoral calcinosis. See tumoral of, 134 rhabdomyosarcoma, 30, 51, 52–53, 56, calcinosis rectal gastrointestinal stromal tumor (GIST), 550–559, 896, 898, 904, 1036. See pulmonary adenocarcinoma, 51, 141 499 also alveolar rhabdomyosarcoma; pulmonary carcinoma, 80 recurrent digital fibroma, 261–264 embryonal rhabdomyosarcoma pulmonary chondroid hamartoma, 137 recurrent orbital malignant meningioma, 750 with anaplasia, 556 pulmonary chondroma, 862 recurrent parapharyngeal rhabdomyoma, 549 botryoid-embryonal rhabdomyosarcoma, pulmonary clear cell “sugar” tumor, 537–538 Reed’s syndrome, 463 508, 510, 556–559 pulmonary edema, 450 Reichel disease, 864–867 chemotherapy-treated, 547

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1102 INDEX

rhabdomyosarcoma (cont.) doxorubicin treatment, 1061 sclerotic fibroma of skin (circumscribed classification, 550–551 epidemiology storiform collagenoma), 214–215 clear cell rhabdomyosarcoma, 562–564 global, 3 sclerotic lipoma, 409–413 clinical presentation, 551–552 U.S., 2–3 scrotal leiomyoma, 466 differential diagnosis, 444, 569, 1049, 1053 HRAS/KRAS mutations, 128 scrotal smooth muscle hyperplasia, 461 genetics, 568 ifosfamide treatment, 1062 scrotal well-differentiated liposarcoma, 434, immunohistochemistry, 53, 59, 62, 72 immunohistochemistry, 62, 81 435 filament proteins, 566–567 plating efficiency, 106 secretory breast carcinoma, 136 myogenic regulatory proteins, 567–568 sarcomatoid carcinoma (carcinosarcoma), 301, segmental neurofibromatosis, 679 myoglobin, 567 379, 522, 798 seminoma, 50, 80, 854–855 pathology (gross/microscopic), 552–554 sarcomatoid (spindle cell) carcinoma of the septic arthritis (acute purulent synovitis), pleomorphic rhabdomyosarcoma, breast, 841–842 939–940 561–562, 1038 sarcomatoid mesothelioma, 822 serine protease inhibitors (SERPINS). See prognosis/clinical course, 568–569 sarcomatoid pulmonary carcinoma, 843–846 alpha-1-antichymotrypsin; sclerosing rhabdomyosarcoma, 564 sarcomatoid renal cell carcinoma, 849–851 alpha-1-antitrypsin sclerosing spindle cell rhabdomyosarcoma, sarcomatoid squamous cell carcinoma, 380, serous cystadenocarcinoma, 631 364 839 sex cord tumors, 904 spindle cell rhabdomyosarcoma, 302 sarcomatous spindle cell GIST, 494–496 silicone (elastomer) deposition, 948 treatment for, 1064 sarcomeric actin, 53 silicone granuloma, 426, 1018 ultrastructure of, 566 scalp angiosarcoma, 631 sinonasal hemangiopericytoma (SNH), 271, of urinary bladder, 2 scalp meningioma (ectopic meningothelioma), 342, 656–658 variants (unusual), 562–566 745, 746 sinonasal leiomyoma, 469–470 rheumatoid synovitis, 941–942 Scandinavian Sarcoma Group, 1074 sinonasal melanoma, 750 rice bodies, 942 Schwann cell tumors, 1, 60–61, 67 sinonasal meningioma, 748–749 Rosai-Dorfman disease (RDD), 62, 997–998 schwannoma, 50, 62, 63, 73, 75, 80, 514, 631, sinonasal solitary fibrous tumors (SFTs), round cell liposarcoma, 110, 447–450, 451, 1031–1032. See also psammomatous 335–336 453, 1021–1022 melanotic schwannoma sinonasal tract leiomyosarcoma, 476 RT-PCR assays, 146, 147 ancient schwannoma, 1032 sirolimus, 504 bilateral vestibular schwannoma, 144–145, skeletal muscle tumor, 75 S100 protein/other multispecific neural 685 skin adnexal tumor, 50, 74, 80, 739 markers, 59–63 cellular plexiform schwannoma, 684 small cell carcinoma, 57, 904, 1051 cartilage and bone tumors, 61 cellular schwannoma, 682–683 small cell osteosarcoma, 59 CD56, 62 conventional schwannoma, 679–682 small intestinal gastrointestinal stromal tumor CD57, 62–63 clinical features, 679 (GIST), 493, 497 epithelial cells/carcinomas, 62 immunohistochemistry, 680–682 small round blue cell tumor. See also small fat (and its tumors), 61 pathology, gross, 679–680 round cell desmoplastic tumor histiocyte-related cells, 62 microscopic, 680 Ewing sarcoma, 30, 559, 897–906, melanocytes (and their tumors), 60 ultrastructure, 682 1048–1049, 1078 mesenchymal tumors, 61–62 description, 679 extrarenal rhabdoid tumor, 918–922 nerve growth factor receptor (low affinity), differential diagnosis, 387, 504, 1024, 1048 genetics, 896–897 p75, 62 epithelioid schwannoma, 684 neuroblastoma, 56, 58, 836, 906–915 Schwann cells (and their tumors), 60–61 gastrointestinal schwannoma, 686–687 small round cell desmoplastic tumor, salivary gland carcinoma, 62 plexiform schwannoma, 683–684 915–918 salivary gland tumors, 80 psammomatous melanotic schwannoma, types/differential diagnosis, 896 sample preparation, 106 63, 320, 687–691 small round cell desmoplastic tumor, 915–918 sarcoma. See also alveolar soft part sarcoma; with rosettes/neuroblastoma-like features, clinical features, 915 angiosarcoma; 684 cytogenics/molecular biology, 916–917 dermatofibrosarcoma protuberans; sarcoma arising in, 685 definition, 915 desmoplastic small round cell schwannomatosis, 686 diagnostic summary/differential diagnosis, tumor; embryonal sclerosing epithelioid fibrosarcoma, 364 918 rhabdomyosarcoma; epithelioid sclerosing extramedullary myeloid tumor, 441 gross/microscopic description, 915–916 sarcoma; extraskeletal sclerosing hemangioma. See benign fibrous history, 915 chondrosarcoma; extraskeletal histiocytomas (BFHs, immunohistochemistry/electron osteosarcoma; extremity sarcoma; dermatofibroma) microscopy, 916 fibrosarcoma; sclerosing lipogranuloma, 423–425 prognosis/clinical outcome, 917 hemangiopericytoma; Kaposi’s sclerosing (inflammatory) liposarcoma, 204 SMARCB1 (SWI/SNF-related, sarcoma; leiomyosarcoma; sclerosing metaplastic carcinoma, 364 matrix-associated, actin-dependent liposarcomas; lymphangiosarcoma; sclerosing neoplasms, 202 regulator of chromatin, subfamily malignant fibrous histiocytoma; sclerosing rhabdomyosarcoma, 564 B, member 1) inactivation, 142 malignant peripheral nerve sheath sclerosing spindle cell GIST, 494 smooth muscle hamartoma of skin, 460–461 tumor; osteogenic sarcoma; sclerosing spindle cell rhabdomyosarcoma, smooth muscle myosin, 53 rhabdomyosarcoma; synovial cell 364 smooth muscle tumors, 52, 54, 75, 77 sarcoma; synovial sarcoma; truncal sclerosing well-differentiated liposarcoma, angioleiomyoma, 463–466 sarcoma 436 angiomyomatous hamartoma of lymph, definition, 2 sclerotic benign fibrous histiocytoma, 215 461

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Index 1103

benign metastasizing leiomyoma, 481 solitary reticulohistiocytoma (solitary surgical management of GIST, 1066 epithelioid tumors of the gynecologic epithelioid histiocytoma), 230 advanced GIST, 1066 tract, 479 sorafenib, 1078 treatment of primary disease, 1066 Epstein-Barr virus–associated spectral karyotyping (SKY), 108, 113 surgical management of retroperitoneal leiomyosarcoma, 485–486 spinal cord associated lipoma, 398 sarcoma, 1065–1066 estrogen-receptor positive smooth muscle spindle cell angiosarcoma, 631, 633 locally recurrent retroperitoneal sarcoma, tumors, 477–478 spindle cell carcinomas, 53 1066 gastrointestinal leiomyoma, 466–469 spindle cell fibroblastic tumors, 48 radiation treatment, 1065 genitourinary tract leiomyoma, 470 spindle cell hemangioma, 402–403, 593–598 subtypes/goals of surgery, 1065 hamartoma of skin, 460–461 spindle cell lipoma, 224, 405–409, 1019, surgical management of soft tissue sarcoma intravenous leiomyomatosis, 480–481 1021–1022 background information, 1057 leiomyoma of male external genitalia, 466 with abundant myxoid matrix, 1021–1022 biopsy/clinical evaluation, 1057–1058 myxoid uterine tumors, 479–480 clinical features, 405–407 reconstructive options, 1058–1059 peritoneal leiomyomatosis, 481–483 differential diagnosis, 409, 1019 sarcoma resection principles, 1058 pilar leiomyoma (piloleiomyoma), 463 genetics, 409 site-specific surgical therapy scrotal smooth muscle hyperplasia, 461 pathology, 407–408 amputation, role of, 1060 sinonasal leiomyomas, 469–470 spindle cell liposarcoma, 435, 438 chemotherapy for histologic subtypes, soft tissue/visceral leiomyosarcomas spindle cell rhabdomyosarcoma, 302, 551, 1064–1065 (excluding uterine), 470–477 555–556 chemotherapy in extremity/truncal of uncertain malignant potential spindle cell synovial sarcoma, 341 sarcomas, 1061–1063 (STUMP), 484–486 splenic angiosarcoma, 628–629 extremity/truncal subtype strategies, uterine leiomyosarcoma, 483–485 splenic hemangioma, 629 1059 soft tissue epithelioid hemangioendothelioma squamous carcinoma, 73, 79 multimodality treatment for subtypes, (EHE), 620 squamous cell carcinoma, 836, 839–841 1063–1064 soft tissue gliomatosis, 741–742 SS18-SSX2 fusion gene, 147 primary surgical therapy, 1059–1060 soft tissue lipoma, 393–397 SS18-SSX fusion genes, 133–134, 146, radiation, role of, 1060–1061 soft tissue tumors 147 surgical staging system (Enneking), 6 classification, 1 SS18L1/SSX1 fusion gene, 147 symplastic glomus tumor, 653 definition, 1 staging systems, 6–7 synaptophysin, 56–58 epidemiology, 2–3 for radiologic evaluation, 17–18 synovia and tendon pathology etiology, 3 surgical (Enneking), 6, 18 acute purulent synovitis, 939–940 histogenesis, 1–2 steroid cell tumors, 64 alkaptonuric ochronosis, 950–951 specific imaging appearances, 21–37 steroid lipomatosis, 401 bone cement, 947–948 angiomatous lesions, 25–30 Stewart-Treves syndrome, 1063 calcium pyrophosphate deposition disease, “cystic” masses, 34–36 stromal tumors, 54 946–947 lipomatous lesions, 21–25 adult granulosa cell tumor, 523–524 carbon material, 948–950 neurogenic tumors, 30–34 aggressive angiomyxoma, 514–518 gouty arthritis, 946 soft tissue/visceral leiomyosarcomas angiomyofibroblastoma, 512 granulomatous synovitis, 944–946 (excluding uterine), 470–477 carcinosarcoma, 520–523 Lyme disease, 940–941 solid epithelial mesothelioma, 819 cellular angiofibroma/male metal fragment deposition, 950 solitary cutaneous neurofibroma, 669 angiomyofibroblastoma-like tumor, polyethylene wear particles, 947 solitary epithelioid histiocytoma, 289, 514–515 rheumatoid synovitis, 941–942 1004–1005 endometrial stromal sarcoma, 518–520 silicone (elastomer) deposition, 948 solitary fibrous tumors (SFTs), 76, 182, endometriosis, 524–526 synovia in osteoarthritis, 942 335–341, 520. See also genital (vaginal) rhabdomyoma, 511–512 synovial biopsy, 939 intra-abdominal solitary fibrous mesodermal stromal polyp, 508–510 synovial fluid analysis, 939 tumors; meningeal solitary fibrous superficial cervicovaginal synovial villous hyperplasia, 942–944 tumors; oral solitary fibrous myofibroblastoma, 510–511 tendon rupture, 951 tumors; orbital solitary fibrous stromatosis. See endometrial stromal sarcoma tenosynovial giant cell tumor, 951–952 tumors; pleural-thoracic solitary STUMP diagnosis, 484–486 atypical/malignant, 940–961 fibrous tumors; sinonasal solitary subcutaneous and intramuscular diffuse type, extra-articular, 958–960 fibrous tumors leiomyosarcoma, 474–475 intraarticular, 957 clinical features subcutaneous pseudosarcomatous fibromatosis localized type, 952–957 in peripheral soft tissues, 337 (fasciitis). See nodular fasciitis synovia in osteoarthritis (degenerative joint at serosal/visceral sites, 335–337 subungual glomus tumor, 648 disease), 942 differential diagnosis, 337–341, 364, 444, sunitinib/sunitinib malate, 504, 1066, 1071, synovial cell sarcoma, 1063 514 1078 synovial chondromatosis, 11, 19–20, 21, 37, genetics, 341 C-kit mutation and response of, 864–867 giant cell angiofibroma variant 1071–1072 clinical features, 864–865 clinical features, 341–342 superficial acral fibromyxoma (SAF), genetics, 867 pathology, 342 321–323 imaging, 865 history/terminology, 335 superficial angiomyxoma (SAM), 314–320 pathology, 865–867 immunohistochemistry, 77–79, 337–341 superficial cervicovaginal myofibroblastoma, synovial chondrometaplasia, 864–867 pathology, 337 510–511 synovial cysts, 34, 35 vs. desmoid, 251 superficial vascular pseudoaneurysm, 603–605 synovial gout, 946

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1104 INDEX

synovial lipomatosis (lipoma arborescens), thymolipoma, 399 inflammatory myofibroblastic tumors, 298 397–398 thyroid carcinoma, 81, 854 solitary fibrous tumors, 337 synovial osteochondromatosis, 864–867 thyroid papillary carcinoma, 131 urothelial (transitional cell) carcinoma, 851 synovial osteochondrosis, 864–867 tissue procurement (for special studies), 8–9 urticaria pigmentosa, 80 synovial sarcoma (SS), 2, 11, 19–20, 30, 51, cytogenetics/cell culture, 8 uterine adenocarcinomas, 72 110, 777–787, 904, 1046–1047 frozen tissue, 8 uterine adenomatoid tumors, 811 clinical features, 778–779 special fixatives, 8–9 uterine carcinosarcoma, 521 demographics, 777–778 TOP2a (DNA alpha 2 topoisomerase), 441 uterine inflammatory myofibroblastic tumors differential diagnosis, 342, 787, 1035, tophaceous pseudogout, 877–879 (IMTs), 298 1047, 1048 trabecular carcinoma of the skin, 834–838 uterine leiomyoma, 79, 137, 477, 478, 539 genetics, 785–787 transport of tumor specimens, 105–106 uterine leiomyomata, 463 immunohistochemistry, 75, 76, 77–79, traumatic neuroma (amputation neuroma), 30, uterine leiomyosarcoma, 84, 475, 483–485 783–785 32, 662 uterine perivascular epithelioid cell tumors pathology, 779–780 trichodiscoma, 317 (PEComas), 538–539, 542 biphasic type, 780, 787 trichogenic myxoma, 317 uterine smooth muscle tumors, 77, 79 monophasic spindle cell type, 780–783, Triton tumor, 712 787 troglitazone for liposarcoma, 432 vaginal mucosal polyps, 508 poorly differentiated type, 783, 787 truncal sarcoma, 1059 vaginal rhabdomyoma, 510 prognostic factors, 783 tryptase, 997 valvular lipomas, 399 SS18-SSX fusion genes in, 133–134, 146, tuberous sclerosis complex (TSC), 531 vascular endothelial growth factor receptor 3 147, 779 tuberous xanthoma, 971 (VEGFR3), 51 SS18-SSX2 fusion genes in, 147 tubulopapillary malignant mesothelioma, vascular leiomyoma. See angioleiomyoma vs. palmar/plantar fibromatosis, 240 818–819 vascular neoplasms, 25. See also angiosarcoma; synovial villous hyperplasia associated with tufted angioma, 578 hemangioendothelioma; joint bleeding (hemosiderotic tumor necrosis factor (TNF), 1075 hemangiopericytoma synovitis), 942–944 tumor suppressor genes, 127 vasculogenesis, 575 systemic calcinosis, 879 tumoral calcinosis, 11, 875–877 vena cava leiomyosarcoma, 476 clinical features, 875–877 venous hemangioma, 575, 583–585 T-cell (NK/T)-cell lymphoma, 992 genetics, 877 verrucous hemangioma, 582–583 T-cell lymphoblastic lymphomas, 177–178, imaging, 877 verruga peruana, 602–603 77–79, 137 pathology, 877 villous synovial hyperplasia, 960 targetoid hemosiderotic hemangioma, Turner syndrome, 585, 606 vimentin, 81 587–589 “two-hit hypothesis” (Alfred Knudson), 127, vincristine, 1064, 1079. See also CyVADIC taxanes, 1078 143, 144–145 vinorelbine, 1078 telangiectasia, 575, 598–600 tyrosinase enzyme glycoprotein (of 75kD), vinyl chloride, 4 port-wine stain, 600 64 viruses, 3 venous lake, 600 tyrosine kinases (TKs), oncogenic activation visceral lipoma, 398–399 tendinous xanthoma, 971 of, 134 Von Hippel-Lindau disease (VHL), 756–757 tendon rupture, 951 von Willebrand factor (vWF, Factor tendon sheath fibromas, 52, 214 UICC-TNM staging system, 6 VIII-related antigen), 49–50, 579, clinical features, 214 ultrasound, 15–17 623, 634 differential diagnosis, 214 of neurogenic tumors, 30–33 vulval lymphedema, 518 genetics, 214 ultrastructure vulval polyps, 508 pathology, 214 adrenal pheochromocytoma, 761 vulvar angiomyofibroblastoma, 54, 80, 478, tenosynovial giant cell tumor (giant cell tumor alveolar soft part sarcoma, 935 512 of tendon sheath), 58, 68, 383, branchial anlage mixed tumor, 804 vulvar leiomyoma, 478 951–952 fetal rhabdomyoma, 547 atypical/malignant, 940–961 glomus tumor, 654–655 WAGR syndrome, 143 differential diagnosis, 960 hemangiopericytoma, 52–53, 77–79, well-differentiated angiosarcoma, 50, 631, 634 diffuse type 343–345 well-differentiated lipoma, 398–399 extra-articular, 958–960 leiomyosarcoma, 474 well-differentiated lipoma-like liposarcoma, intraarticular, 957 malignant mesothelioma, 825 536 genetics, 959–960 MPNST, 711 well-differentiated liposarcoma (WDLS), 83, immunohistochemistry/histogenesis, 959 neurofibromas, 677 398, 402, 434–441, 447, 1021, localized type, 952–957 rhabdomyosarcoma, 566 1059. See also inflammatory testicular adnexa, 476 schwannoma, 682 (lymphocyte-rich) testicular calcifying Sertoli cell tumor, 320 umbilical polyp, 258–259 well-differentiated liposarcoma; testicular Leydig cell tumors, 64 undifferentiated (homologous) lipoma-like liposarcoma; sclerosing tetraspanin 31/SAS (sarcoma amplified carcinosarcoma, 521 well-differentiated liposarcoma; sequences) gene, 441 undifferentiated pleomorphic sarcoma, 561, spindle cell liposarcoma TFE3 gene, 134 1030–1031 clinical features, 434–435 thoracic paraganglioma, 767 undifferentiated sarcoma, 566 differential diagnosis, 396, 414, 441, 448, thorium oxide deposition (thorotrast), urinary bladder leiomyosarcoma, 476 451, 1017 974–976 urinary bladder pheochromocytoma, 761–767 genetics, 441 thorothrast, 4 urinary bladder/urogenital tract HMGA2 in, 138 thrombomodulin (CD141), 51 adenocarcinoma, 72, 401 immunohistochemistry, 438–441

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Index 1105

myxoid components in, 438 wide excision (WE), 1060, 1063–1064 xanthelasma (planar xanthoma), 971–972 pathology, 435–438 Wilms’ tumor, 54, 56, 59, 81, 896. See also xanthogranulomatous inflammation, 970–971 well-differentiated papillary mesothelioma, extrarenal Wilms’ tumor; WAGR xanthoma, 971–972 813–816 syndrome xanthomatous pseudotumor, 297 well-differentiated smooth/skeletal muscle Wilms’ tumor suppressor gene (WT1), tumors, 75 129–131, 142–143 ZIC1 transcription factor, 441

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