Locators in Italics Refer to Figures. AAM See Aggressive

Home , Aggressive angiomyxoma, Microvenular hemangioma

Cambridge University Press 978-1-107-56727-6 — Modern Soft Tissue Pathology 2nd Edition Index More Information

Index

Locators in italics refer to Figures.

AAM see aggressive adenocarcinoma see also aggressive digital papillary appearance 896, 896 angiomyxoma carcinoma adenocarcinoma clinical features abdominal endometriosis 506–507 aggressive digital papillary 764–765, 765, 766 soft tissue 895 histopathology 507, 508 764–765, 765–766 aggressive fibromatosis see visceral sites 895–896 abdominal ependymoma keratins 65 desmoid fibromatosis cytology 1005, 1005–1006 711–713, 713 pancreatic 809 AIDS see HIV/AIDS differential diagnosis 737, 900 abdominal inflammatory pulmonary 792–793, 805, 806 AJCC staging system 5 electron microscopy 899, 899 myofibroblastic tumor adenofibroma 505 AKT/mTOR signaling pathway genetics 129, 900 (IMT) 289 adenomatoid tumor 777, 778 128, 136 histogenesis 899–900 epithelioid variant 290, 291 adenomatous polyposis coli gene inhibitors 133, 518 histopathology 896–898, abdominal wall desmoid tumor (APC) 137–138, 245–246 ALCL see anaplastic large cell 897–898 239–241 adenosarcoma 505 lymphoma immunohistochemistry abscesses 33–34 adipose tissue pathology see also alcohol fixation 8 898–899, 899 aCGH (array-CGH) 100–101, lipoblastoma; ALK/ALK (anaplastic lymphoma metastatic disease 1027 123, 143 lipodystrophy; kinase) American Joint Committee of acquired elastotic hemangioma lipogranuloma; lipoma; in ALCL 952–954 Cancer (AJCC) staging 575, 577 lipomatoses; liposarcoma as an IHC marker 70, 291 system 5 acquired progressive cytology 977–982 gene fusion 124, 127, 292, AML see angiomyolipoma lymphangioma (benign imaging 19–25 953–954 amplification of genes 98–99, lymphangioendothelioma) immunohistochemistry 46, 57, in inflammatory 101, 130–131, 611, 585–587 72 myofibroblastic tumor 875–876 histopathology 586, 586–587 adrenal gland 127, 291–292 amputation 1015 acquired tufted angioma cortical tumors in neuroblastoma 130–131, 877 amputation (traumatic) neuroma 566–567, 568 carcinoma 60, 815, 816 alkaptonuric ochronosis 913, 914 30, 639, 640 acral arteriovenous tumor myelolipoma 390, 391 allele-specific PCR (AS-PCR) 141 amyloid tumor (amyloidoma) (arteriovenous medullary tumors see alpha smooth muscle actin 50, 546 936–938, 937–938 hemangioma) 26, pheochromocytoma Alport syndrome 450 anaplastic large cell lymphoma 561–562, 562 adult fibrosarcoma 336, 351–352 ALT (atypical lipomatous tumor) (ALCL) acral myxoinflammatory cytology 984, 985 see well-differentiated noncutaneous (systemic) fibroblastic sarcoma spindle cells 352–353 liposarcoma 952–954, 953 352–356 adult granulosa cell tumor 505–506 altitude (and carotid body primary cutaneous 954 appearance 354 appearance 506 paraganglioma) 735 anaplastic lymphoma kinase see cytology 984, 985 histopathology 505–506, 506 alveolar rhabdomyosarcoma ALK/ALK differential diagnosis 354–355, immunohistochemistry 506, 507 (alveolar sarcoma) anaplastic rhabdomyosarcoma 984 adult myofibroma 267, 270–271 539–541 543–544, 544 histopathology 354, 355 adult rhabdomyoma 529,529–530 cytology 994, 994 anaplastic spindle cell pseudolipoblasts 356 adults with “pediatric” sarcomas genetics 129, 134–135, rhabdomyosarcoma 542 Reed–Sternberg-like cells 356 1031 539–541, 542, 878–879 ancient hematoma see organizing acroangiodermatitis (pseudo- AFH see angiomatoid fibrous histopathology 539, hematoma Kaposi’ssarcoma)580, 580 histiocytoma 540–541 ancient schwannoma 655, actins 49–50, 546 AFX see atypical fibroxanthoma leukemoid variant 540 989–990, 990 acute myeloid leukemia 954 age, and tumor type 19 imaging 539 aneuploidy 98–99 ACVR1 (activin-like receptor) aggressive angiomyxoma (AAM) immunohistochemistry 546, hyperdiploidy 548, 876–877 845 female 498–501 547 trisomy 237, 246, 294, 506, adamantinoma 770, 770 appearance 498, 499 mixed with embryonal form 544 921 adamantinoma-like Ewing histopathology 498–499, 500 alveolar soft part sarcoma aneurysmal bone cyst 104, sarcoma 99 male 498 (ASPS) 895–900 844–845

1036

Index

aneurysmal fibrous histiocytoma cytology 1000–1001, 1002 in lymphedematous B-cell lymphoma 224–225, 224 differential diagnosis 517 extremities 4, 603–604 Burkitt lymphoma 949, 950 angioblastic meningioma genetics 517–518 in nerve sheath tumors 607, and crystal-storing histiocytosis (meningeal SFT) 324–325, histopathology 515–517, 515 659, 683 964–965, 965–966 331 epithelioid 517, 517 postradiation 603 diffuse large 947, 948 angioblastoma of Nakagawa lipomatous component 515, rare sites 607 extranodal marginal zone (acquired tufted 515–516 splenic 606 946–947, 947 angioma) 566–567, 568 smooth muscle component treatment 1018 follicular 945–946, 946 angioendothelioma see papillary 516–517, 516 angulated bodies 673 intravascular 948–949, 949 intralymphatic vascular component 516,516 anoctamin-1 484–485 lymphoblastic 947–948, 948 angioendothelioma immunohistochemistry 59, 59, anthracyclines see doxorubicin plasmablastic 949–950, 951 angioendotheliomatosis, reactive 60, 61, 517 Antoni A/Antoni B areas in bacillary angiomatosis 578–579 579–580 angiomyoma (angioleiomyoma) schwannoma 655 histopathology 578, 579 angiofibroma, cellular (vulvar) 446–447 aorticopulmonary Warthin–Starry stain of 496–497, 497–498 histopathology 446–447, paraganglioma 734 Bartonella 579 angiofibroma, giant cell 330, 331 448–449 APC (adenomatous polyposis BAF47 protein 74, 133–134 see angiofibroma, juvenile angiomyomatous hamartoma of coli) 137–138, 245–246 also INI1/INI1 nasopharyngeal 263–264, lymph node 444, 445 appearance see gross anatomy/ (SMARCB1) 263–265 angiomyxoma, aggressive morphology Baker (popliteal) cyst 17,31–32, 33 angiogenesis 553 female 498–501 appendiceal GISTs 481 BAP1/BAP1 (BRCA1-associated angiography 13, 29 appearance 498, 499 array-CGH (aCGH) 100–101, protein) 794 angiokeratoma 559–560, 561 histopathology 498–499, 500 123, 143 Bartonella infections angioleiomyoma 446–447 male 498 arsenic 4, 798 bacillary angiomatosis histopathology 446–447, angiomyxoma, superficial arteriovenous hemangioma 26, 578–579, 579 448–449 (cutaneous myxoma) 561–562, 562 verruga peruana 579 angiolipoma 388–390 306–310 arthritis see also pseudogout Warthin–Starry stain 579 histopathology 389, 389 clinical features 306–307, 309 gout 841, 903, 907, 909–910 basal cell keratins 67–68 cellular variant 389–390, 390 differential diagnosis osteoarthritis 905–906, 907 basal cell nevus syndrome (Gorlin infiltrative see intramuscular 307–308 rheumatoid 904–905, 906 syndrome) 529 hemangioma genetics 309–310 septic 903–904, 904 basement membrane proteins 72 angiolymphoid hyperplasia with histopathology 307, 308–309 AS see angiosarcoma BCL2 protein 70 eosinophilia see angiosarcoma (AS) 593, 601–611, asbestos 782–783 BCOR gene 124, 866–867 epithelioid hemangioma 1031 asbestos fibers 782 BCOR-CCNB3 fusion sarcoma angiomatoid fibrous appearance ferruginous bodies 782–783, 866–867, 866–867 histiocytoma (AFH) breast 605 783 Beckwith–Wiedemann 283–288 deep soft tissue 606 Askin’s tumor (chest wall Ewing syndrome 535, 856 appearance 284–285 lymphedematous arm 604 sarcoma) 856 see also Bednar tumor (pigmented histopathology 284–285, 286 scalp 603 Ewing sarcoma family of variant of DFSP) 342, 342 cysts 286 breast parenchyma 604–605 tumors benign fibrous histiocytoma hemorrhagic appearance cardiovascular 606–607 ASPS see alveolar soft part (BFH, dermatofibroma) 286–287 in children 606, 608 sarcoma 220–228 resembling lymph node cutaneous (face and scalp) ASPSCR1 (ASPL)-TFE3 gene appearance 220–221, 221, 224 metastasis 285 602–603 fusion 129, 900 clinical features 220 variations 287–288 cytology 995–997, 998 ATF1/ATF gene fusion 126, 287– cytology 987, 988 angiomatosis 562–564 of deep soft tissues 605, 608 288, 707–708 differential diagnosis 224–226, histopathology 563–564, 565 differential diagnosis 27, 605, atypical decubital fibroplasia 344–345, 987 imaging 27, 29 608–610, 996–997 (ischemic fasciitis) 195 genetics 228 angiomatosis, bacillary 578–579 etiology 3–4, 593 histopathology 195, 197 histopathology 221–222, 222–223 histopathology 578, 579 foreign-body-associated atypical fibrous histiocytoma aneurysmal variant 224, Warthin–Starry stain of 607–608 226–228, 227 224–225 Bartonella 579 genetics 611 atypical fibroxanthoma (AFX) atypical variant 227 angiomatous lesions see in germ cell tumors 607 364–367 cellular variant 225–226 angiomatosis; hepatic 3–4, 606 appearance 365 epithelioid variant 226 angiosarcoma; histopathology 608 histopathology 365–366, 366 lipidized variant 227 hemangioendothelioma; breast parenchyma 605 immunohistochemistry 366, 367 immunohistochemistry hemangioma; epithelioid 608, 609–610 atypical glomus tumor 626, 630 222–224, 225, 227, 223 hemangiopericytoma; hepatic 606 atypical lipomatous tumor (ALT) metastasis 221 lymphangioma lymphoid infiltration 609 see well-differentiated recurrence 220 angiomyofibroblastoma, vulvar poorly differentiated 610 liposarcoma variants 224 494–496, 495–496 radiation-induced 603, 604 atypical mesothelial hyperplasia aneurysmal 224–225, 224 angiomyofibroblastoma-like splenic 607 790 atypical 226–228, 227 tumors in men 496–497, well-differentiated 608 atypical tenosynovial giant cell cellular 225–226, 225 497 imaging 27 tumor 921–922, 922 epithelioid 225, 226 angiomyolipoma (AML) 513–518 immunohistochemistry 45, 66, atypical teratoid/rhabdoid tumor granular cell 228 appearance 514 610–611, 611 882, 885 lipidized 225–226, 227

1037

Index

benign lymphangioendothelioma histopathology 535, 537 histopathology 841, 843 cardiac inflammatory (acquired progressive brachytherapy 1015–1016 imaging 841, 842 myofibroblastic tumor lymphangioma) 585–587 brachyury (transcription factor) calcium pyrophosphate 289, 291, 291 histopathology 586, 586–587 766 deposition disease cardiac lipoma 385 benign metastasizing leiomyoma BRAF mutations 487, 702, 958– see pseudogout cardiac myxoma 313–315 464–465, 465 961 Call–Exner bodies 505 histopathology 314, 314–315 benign Triton tumor brain tumors CALLA (common acute degenerative features 316 (neuromuscular angiosarcoma 289 lymphoblastic leukemia glandular epithelial hamartoma/choristoma) atypical teratoid/rhabdoid antigen, CD10) 70–71 differentiation 314, 316 530, 530, 638, 638 tumor 882, 885 calponin 51 cardiac paraganglioma 734 benign tumors inflammatory myofibroblastic calretinin 72, 752 cardiac rhabdomyoma 523, 523 classification 2 tumor 289 capillary hemangioma 26 see also cardiac synovial sarcoma 746 distinguishing from malignant metastatic small cell infantile hemangioma; Carney complex 306, 308–310, tumors 11–12, 19 carcinoma of the lung lobular capillary 661 epidemiology 3, 11 806 hemangioma Carney triad 482, 825 beta-2 microglobulin-derived branchial anlage mixed tumor acquired elastotic Carney–Stratakis syndrome amyloidosis 938 (ectopic hamartomatous hemangioma 575, 577 482 beta-catenin 128, 138, 631 thymoma) 766–768 acquired tufted angioma carotid body paraganglioma immunohistochemistry of histopathology 768–769, 768 566–567, 568 734–735, 737 desmoids 245 immunohistochemistry 768, cherry hemangioma 575, 576 appearance 735 BFH see benign fibrous 769 imaging 26, 28 histopathology 735–737, 736 histiocytoma breast angiosarcoma 604–605, intramuscular 28, 564 immunohistochemistry 54 biological therapies see targeted 605 microvenular hemangioma carpal tunnel syndrome 938 therapies breast carcinoma 575, 576 Carrion’s disease (verruga biopsy 6–7 metastatic 73, 803–805, 805 carbon fibers/graphite 911–912, peruana) 579 fine-needle aspiration 975, 1012 sarcomatoid 805, 805 912 cartilage-associated lesions 825 of synovia 903 breast fibromatosis 239 carcinogens (chemical) 3–4, 606, see also chondroma of tissue handling 8 breast implants 782–783, 798 soft parts; extraskeletal types of 1012–1013 ALCL 953 carcinoid tumors 53, 806 mesenchymal biphasic mesothelioma 786–787, silicone granuloma 409, 410 carcinoma chondrosarcoma; 787 breast myofibroblastoma adrenocortical 60, 815, 816 extraskeletal myxoid biphasic synovial sarcoma 68, 218–220 aggressive digital papillary chondrosarcoma; 747–748, 747–748 cytology 983, 983–984 adenocarcinoma synovial chondromatosis Birbeck granules 961 histopathology 218–219, 220 764–765, 765, 766 alkaptonuric ochronosis 913, bladder carcinoma 67–68, 811–813 breast smooth muscle breast 914 bladder inflammatory hamartoma 445 metastatic 73, 803–805, 805 differential diagnosis 827, myofibroblastic tumor Brooke–Spiegler syndrome 763 sarcomatoid 805, 805 830–832, 834 (IMT) 288–289, 292 brown fat 398 compared with mesothelioma immunohistochemistry 48, 57 bladder leiomyoma 452 brown fat tumor see hibernoma 792–793, 792–793 lipoma with metaplastic bladder paraganglioma 729–734, Burkitt lymphoma 949, 950 gastrointestinal 808–809 cartilage and bone 733–734 bursal fluid collections 32–33 hepatocellular 809, 809 382–383, 383 blue nevus see cellular blue nevus immunohistochemistry 50, 58, Castleman disease 941–943, 944 bone cement 911, 911 cadherins 70 65, 812, 818–819 catecholamines bone imaging 11, 13 café au lait spots 653 lung neuroblastoma 870 bone-forming tumors 825 see Cajal cells 475, 484 adenocarcinoma/large cell pheochromocytoma 723, 726 also calciphylaxis; calcific uremic arteriolopathy see 792–793, 805, 806 cauda equina paraganglioma fibrodysplasia ossificans calciphylaxis sarcomatoid 807, 808 738–740, 739 progressiva; fibro-osseous calcification see also small cell 53–54, 802–803, cavernous hemangioma 558–559 pseudotumor of the calciphylaxis; tumoral 806, 806–807 appearance 559 digits; metaplastic bone; calcinosis Merkel cell see Merkel cell histopathology 559, 560 myositis ossificans; hemangioma 25 carcinoma imaging 25–26, 26–27 odontogenic myxoma; imaging 11, 13 myoepithelial 50, 702 CBN see cellular blue nevus osteosarcoma, neuroblastoma 871 ovarian 815–816 CCNB3-BCOR fusion sarcoma extraskeletal; phosphaturic mesenchymal pancreatic 809 866–867, 866–867 phosphaturic tumor 836 prostatic 125, 813–815, 814–815 CD10 (CALLA) 70–71 mesenchymal tumor; synovial chondromatosis 34, 35 renal cell see renal cell CD31 (PECAM-1) 41–44, 610 tophaceous pseudogout; synovial sarcoma 749 carcinoma CD34 (hematopoietic progenitor tumoral calcinosis calcified myonecrosis 926 squamous cell 758, 803, 803–804 cell antigen) 44–47 differential diagnosis 836–839, calcifying aponeurotic fibroma thyroid 184, 816–817, 817 CD34-positive fibroblasts 182 842–847, 849 256–257 unknown primary 818–819 dermatofibrosarcoma Borrelia burgdorferi infection histopathology 257, 259–260 urothelial 67–68, 811–813 protuberans 46, 345 Lyme disease 904, 905 calcifying fibrous pseudotumor carcinosarcoma see malignant GISTs 47, 485, 485–486 lymphoma 952 253–254 mixed müllerian tumor; superficial acral fibromyxoma botryoid rhabdomyosarcoma histopathology 254, 255 sarcomatoid carcinoma 312 533–534 calciphylaxis 840–841 cardiac angiosarcoma 606–607 CD56 (NCAM) 56, 806

1038

Index

CD57 (Leu7) 56 Charcot joint 906 inflammatory myofibroblastic chondroma of soft parts 825–827 CD68 62 chemoradiotherapy 1028–1029 tumor see inflammatory appearance 826, 826 CD99 71–72, 71, 861–862, 862, chemotherapy see also targeted myofibroblastic tumor histopathology 826–827, 826 865, 876 therapies juvenile hyaline fibromatosis imaging 825, 826 CD117 (KIT) adjuvant/neoadjuvant 253, 269–272, 271–272 chondromatosis, synovial 825, immunohistochemistry 74, 1017–1018, 1027–1028, juvenile nasopharyngeal 827–830 484, 485, 611 1028–1029 angiofibroma 263–265 appearance 827–828, 828 mutations for advanced/metastatic juvenile xanthogranuloma 63, histopathology 828–830, 829 in GISTs 131–132, 475, 486– disease 1029–1030 224, 961–962, 962–963 imaging 34, 35, 827, 828 487 high-dose therapy 1030 Kaposi’s sarcoma 612–613 secondary 827, 829, 829–830 in mastocytosis 956 and hyperthermia 1028 Langerhans cell histiocytosis chondrosarcoma see extraskeletal and TKI sensitivity 486, limb perfusion therapy 1028 960–961, 960–961 mesenchymal 1024–1026 response by histologic type/ leiomyosarcoma 459 chondrosarcoma; NGS profile 145 anatomic location lipoblastoma 19, 21, 405–407, extraskeletal myxoid as a therapeutic target 132, 1030–1032 406–407, 978, 979 chondrosarcoma 487–488 rhabdomyosarcoma and lipofibromatosis 257–260, CHOP (DDIT3) gene fusion CD163 62 Ewing sarcoma 547–548, 260–261, 283 126–127, 127, 434–435 CDK4/CDK4 (cyclin-dependent 1018–1019 lipoma (spinal cord) 384–385 chordoma 765–766 kinase 4) 101, 134 in adults 1031 liposarcoma 430 appearance 766 CDKN1a (cyclin-dependent cherry hemangioma 575, 576 lymphangiomatosis 587, 587– histopathology 765–766, 767 kinase inhibitor 1a) (p21) chief cells 723 588 immunohistochemistry 66, 135 childhood tumors 19, 249, 277 melanotic neuroectodermal 766, 767 CDKN2a/CDKN2b (cyclin- angiomatoid fibrous tumor of infancy 708– choristoma, neuromuscular 530, dependent kinase histiocytoma 283–288 709, 710, 991, 991 530, 638, 638 inhibitors 2a/2b) angiosarcoma 606, 608 mortality 855 chromaffin paraganglioma see (p14ARF/p15a/p16) 77, calcifying aponeurotic fibroma nasal glioma 710 pheochromocytoma 134–135, 682 256–257 neuromuscular choristoma chromogenic in situ hybridization cDNA microarrays 143–144 calcifying fibrous pseudotumor 530, 530, 638, 638 (CISH) 99–100 cell culture media 93 253–254, 255 neurothekeoma 224, 277–282, chromogranin A 54 for transport 93 cellular plexiform 279–281 chromosomes see cytogenetics cell cycle markers 75–77 schwannoma 657 papillary intralymphatic chromothripsis 101–102 cell cycle regulatory pathways chemotherapy 547–548, 1018– angioendothelioma 593– CIC-DUX4 fusion sarcoma 134–136 1019 594, 593–595 865–866, 866, 866 cellular angiofibroma (vulvar) cranial fasciitis 182, 249–251, plexiform fibrohistiocytic circumscribed storiform 496–497, 497–498 250 tumor 224, 281–283, 368 collagenoma (sclerotic cellular angiolipoma 389–390, 390 fibrodysplasia ossificans Proteus syndrome 272–273, fibroma) 211, 214 cellular blue nevus (CBN) 694–695 progressiva 845, 846 272 cirsoid aneurysm (arteriovenous atypical/malignant 694–695 fibromatosis colli 251, 251–252 rhabdoid tumor see rhabdoid hemangioma) 26, differential diagnosis 694–695, fibrous hamartoma of infancy tumor, extrarenal 561–562, 562 707 265–266, 265–266 rhabdomyoma CISH (chromogenic in situ histopathology 694, 695 fibrous umbilical polyp cardiac 523, 523 hybridization) 99–100 malignant 696 251–253, 252 fetal 528, 528–529 citric acid cycle dysfunction 138, immunohistochemistry 59, 61, Gardner fibroma 207, 262, rhabdomyomatous 724–725 694 262–263 mesenchymal hamartoma classification cellular fibrous histiocytoma giant cell fibroblastoma 528–530, 530 hemangioma 554 225–226, 225 342–343, 343, 344, 344, rhabdomyosarcoma see myxofibrosarcoma 356–357 cellular neurofibroma 645–646, 343 rhabdomyosarcoma neuroblastoma 867–870, 873, 646, 652 gingival fibromatosis 253, 253– smooth muscle hamartoma of 874 cellular plexiform schwannoma 254 skin 443–444, 444 paraganglioma 723 657 gingival granular cell tumor CHK2 (checkpoint kinase 2) 135 rhabdomyosarcoma 531, 993 cellular schwannoma 655–656, 657 676, 677 chloroma (extramedullary skeletal muscle tumors central nervous system tumors gliomatosis 710–711, 711 myeloid tumor) 954 527 see brain tumors; spinal grading system 4–5 histopathology 954–955, 954 soft tissue tumors 1–2 tumors infantile diffuse fibromatosis immunohistochemistry 954, clear cell renal carcinoma 810 centromere-specific FISH probes 260–261, 261 955 clear cell rhabdomyosarcoma 97 infantile digital fibroma sclerosing 424 544, 544 cerebriform fibrous proliferation 254–256, 256, 256, 257 choanal polyps 265 clear cell sarcoma, in Proteus syndrome 273 infantile fibrosarcoma 292– chondro-osseous metaplasia in gastrointestinal 708 cervicovaginal myofibroblastoma 294, 293–294 lipoma 382–383, 383 histopathology 708, 709 493 infantile hemangioma (and chondroid lipoma 402–404 clear cell sarcoma of tendons and histopathology 493, 493 related forms) 554–557, appearance 402 aponeuroses 704–708 immunohistochemistry 493, 494 556 histopathology 402–403, 402– cytology 1006, 1006 CGH (comparative genomic infantile myofibroma/ 403 differential diagnosis 702, 707, hybridization) 100–101, myofibromatosis 266– chondroid syringoma see mixed 1006 123, 143 268, 269–270, 984, 984 tumor genetics 707–708

1039

Index

clear cell sarcoma of tendons and gadolinium-associated fibrosis CDKN2a/CDKN2b (p14ARF/ epithelioid aponeuroses (cont.) 936, 936 p15a/p16) 77, 134–135, 682 hemangioendothelioma histopathology 705–707, 706– thorotrast reaction 3, 934–935, CYLD gene 763 997, 998 707 935 cylindroma 763–764, 764 epithelioid sarcoma immunohistochemistry 56, 59, copy number variation 98–99, cystic hygroma 582, 584 1003–1004, 1004 707, 708 143 see also amplification cysts/cystic masses see also Ewing sarcoma/PNET clear cell “sugar” tumor 519–520 of genes multicystic peritoneal 1004–1005, 1005 histopathology 519, 520 core biopsy 6, 1012 mesothelioma fat necrosis 977–978, 978 clonal evolution 94, 123, 145 cotton fiber granuloma 934, 935 Baker (popliteal) cyst 17,31– fibromyxoid sarcoma (low- clonality assay (HUMARA) Cowden’s disease 211 32, 33 grade) 986–987, 986 138–139 cranial fasciitis 182, 249–251 cutaneous myxoid cyst fine-needle aspiration 975 CNS tumors see brain tumors; histopathology 250, 250–251 305–306, 307, 306 glomus tumor 998, 999 spinal tumors imaging 250 ganglion cyst 32, 33, 303–305, granular cell tumor 1002, 1002 COBRA-FISH (combined binary cranial nerve schwannoma 133, 304–305 hibernoma 978–979, 980 ratio FISH) 100 654, 659–660 imaging 14, 31–34 leiomyosarcoma 995, 996 coccygeal glomus bodies 624 CREB1-EWSR1 gene fusion 126, schwannoma 656 lipoblastoma 978, 979 COL1A1-PDFGB gene fusion 287–288, 708 cytogenetics 92, 976–977 lipoma 977, 977 128–129, 345 CREB3L1/CREB3L1 127, 348–349 CGH/aCGH 100–101, 123, 143 myxolipoma 977, 977 collagenase 93 CREB3L2/CREB3L2 126–127, chromosomal abnormalities pleomorphic/spindle cell collagenous fibroma 208–209 348–349, 351 amplification 98–99, 101, 978, 979 appearance 208, 210 crizotinib 127 130–131, 611, 875–876 liposarcoma histopathology 208, 210–211 Crohn’s disease 907 fusion see fusion genes myxoid/round cell 981–982, immunohistochemistry 208, crystal deposition disease see hyperdiploidy 548, 876–877 981 211 pseudogout multiple/complex 103, 108– well-differentiated 979–981, collagens 182 crystal-storing histiocytosis 964– 110, 123, 134 980 collagen IV 72 965, 965–966 translocations 103–108, 117, malignant fibrous in palisaded myofibroblastoma CSF1 gene translocation (colony 120 histiocytoma 216, 218, 219 stimulating factor 1) 914, trisomy 237, 246, 294, 506, 921 giant cell 989 colon 920–921 conventional 8 pleomorphic 988, 988 carcinoma 808 CT see computed tomography cell culture and harvest 8, malignant peripheral nerve FAP 137–138 CTNNB1 (beta-catenin gene) 93–94 sheath tumor 992,992–993 GIST 480–481 128, 138, 246, 631 G/GTW-banding stains 94 melanotic neuroectodermal leiomyoma 451–452 culture media 93 image analysis 94 tumor of infancy 991 leiomyosarcoma 457 for transport 93 pros and cons 95 meningioma 990–991, 991 lipoma 385 cutaneous anaplastic large cell specimen preparation 93 mesenchymal nerve sheath tumor 661, 662 lymphoma 954 specimen selection 92 chondrosarcoma, PEComa 521, 522 cutaneous angiosarcoma 602– specimen transport 92–93 extraskeletal 1000, 1000 colony stimulating factor 1 gene 603 FISH 95 myoepithelioma/mixed tumor (CSF1) 914, 920–921 appearance 603 in Ewing sarcomas 97, 864 cytology 1000, 1001 combined binary ratio FISH histopathology MYCN amplification 98–99, myofibroblastoma, breast 983, (COBRA-FISH) 100 epithelioid 609 877 983–984 comparative genomic lymphoid infiltration 609 probes 97–99, 99–100, 119 myositis ossificans 999, 999– hybridization (CGH) poorly differentiated 610 pros and cons 102 1000 100–101, 123, 143 well-differentiated 608 specimen preparation 98, myxofibrosarcoma 985–986, composite rhabdoid tumor 882 cutaneous follicular lymphoma 140–141, 976 986 computed tomography (CT) 13, 25 945–946 specimen selection 95–97 myxoid chondrosarcoma, cavernous hemangioma 26 cutaneous leiomyosarcoma technical variations 99–100 extraskeletal 1006, 1007 chondroma of soft parts 826 455–456, 456 nomenclature 94–96 myxoinflammatory fibroblastic extraskeletal myxoid cutaneous mucinosis, focal 310, 311 cytokeratins see keratins sarcoma 984, 985 chondrosarcoma 830 cutaneous myxoid cyst 305–306, cytokines 362 myxoma, intramuscular 1000, fibrodysplasia ossificans 846 306, 307 cytology 1001 lipomatous lesions 19, 23 cutaneous myxoma see adult fibrosarcoma 984, 985 myxopapillary ependymoma lymphangioma 28 superficial angiomyxoma alveolar soft part sarcoma 991–992, 992 myositis ossificans 12, 843 cutaneous neurofibroma 1005, 1005–1006 neurofibroma 990, 990 synovial chondromatosis 34, 35 644–645 ancillary techniques 975–977 nodular fasciitis 982, 982 tumor within a tumor 1025 appearance (NF1 patient) 653 angiomyolipoma 1000–1001, rhabdoid tumor, extrarenal congenital fibromatosis see histopathology 645, 645–646 1002 1007–1008, 1008 infantile myofibroma/ cutaneous PEComa 522 angiosarcoma 995–997, 998 rhabdomyoma 993, 993 myofibromatosis cutaneous pleomorphic benign fibrous histiocytoma rhabdomyosarcoma 993–994, congenital fibrosarcoma see liposarcoma 435 987, 988 994–995 infantile fibrosarcoma cutaneous sarcomatoid clear cell sarcoma 1006, 1006 schwannoma 989, 989 congenital hemangioma 556–557 carcinoma 803–804, 803 desmoid fibromatosis ancient 989–990, 990 congenital self-healing cyclin-dependent kinase 982–983, 983 synovial sarcoma 1003, 1003 reticulohistiocytosis 960 inhibitors (CDKNs) 76–77 desmoplastic small round cell tenosynovial giant cell tumor contrast media 13–14 CDKN1a (p21) 135 tumor 1006–1007 987, 987

1040

Index

D2–40 (podoplanin antibody) 47 differential diagnosis 224, 344– desmoplastic small round cell DUX4-CIC fusion sarcoma Dabska tumor see papillary 345 tumor (DSRCT) 879–882 865–866, 866, 866 intralymphatic genetics 128–129, 345 clinical features 882 dysgerminoma 818 angioendothelioma giant cell fibroblastoma cytology 1006–1007 dacarbazine 1030 342–343 differential diagnosis 806, 808, E-cadherin 70 DDIT3 (CHOP) gene fusion histopathology 338, 338–340, 881–882 ear see also vestibular 126–127, 127, 434–435 340 genetics 126–156, 881 schwannoma deciduoid mesothelioma 785– Bednar tumor 342 histopathology 879–880, 880 jugulotympanic 786, 786 borders 339 immunohistochemistry 75, paraganglioma 737, 738 dedifferentiated leiomyosarcoma fat infiltration 339 880, 880 meningioma 716 453–454, 454 fibrosarcomatous DFSP see dermatofibrosarcoma EBV see Epstein–Barr virus dedifferentiated liposarcoma transformation 341–342, protuberans eccrine acrospiroma (DDLS) 7, 416, 424–430 341 diabetic scleroderma 207 (hidradenoma) 762, 763 appearance 425, 426 giant cell fibroblastoma diagnosis 6–9, 17–19 see also eccrine spiradenoma 762–763, clinical features 424–425 342–343, 343–344 imaging; 764 differential diagnosis 424, 429– myoid nodules 341 immunohistochemistry ectomesenchymoma 545–546, 430, 487 myxoid variant 340 diffuse angiokeratoma in Fabry 862–863, 863 genetics 108, 430 immunohistochemistry 46, disease 560 ectopic hamartomatous histopathology 425–428 343–345, 345 diffuse extra-articular thymoma (branchial leiomyosarcomatous treatment tenosynovial giant cell anlage mixed tumor) differentiation 429 imatinib 129, 345–346 tumor 919–921, 921 766–768 meningothelial-like whorls surgery 337, 1015 diffuse intra-articular histopathology 768–769, 768 426–427, 427–428 dermatomyofibroma 212, 215 tenosynovial giant cell immunohistochemistry 768, pleomorphic forms 428 desmin 50, 486, 546, 899 tumor 918–921 769 resembling inflammatory desmoid fibromatosis (desmoid appearance 919, 919 ectopic meningioma see MFH 426 tumor) 238–246 histopathology 919, 920 meningioma, ectopic resembling various sarcoma appearance 242, 242 diffuse large cell B-cell EGLN1/PHD2 (prolyl types 427 clinical features 238–240 lymphoma 947, 948 hydroxylase) mutations rhabdomyosarcomatous cytology 982–983, 983 diffuse neurofibroma 648, 651, 726 differentiation 429 differential diagnosis 184–185, 652 see also pigmented EGLN2/PHD1 (prolyl imaging 6, 24, 25 244–245, 487 neurofibroma hydroxylase) mutations immunohistochemistry 429 genetics diffuse symmetric lipomatosis 726 deep (benign) fibrous APC 137–138, 245–246 386–387 EHE see epithelioid histiocytoma 220, 224, 987 CTNNB1 138, 246 digital mucoid cyst 305–306, 306, hemangioendothelioma deep granuloma annulare 927– other 240, 246 307 elastofibroma 204–206 929, 928 histopathology 242, 243–244 digital papillary adenocarcinoma appearance 204–205, 205 deep plexiform schwannoma dilated blood vessels 243 764–765, 765–766 histopathology 205–206, 205– 657 keloid-like fibers 243 dioxin 3 206 deep soft tissue angiosarcoma mesenteric desmoids 245 disseminated xanthoma 932 imaging 22,34 605, 606, 608 skeletal muscle infiltration DNA extraction from formalin- electron microscopy 8, 976 definition of soft tissue tumors 1 244 fixed specimens 140–141 alveolar soft part sarcoma 899, degenerative joint disease imaging 34–35, 36 DNA microarrays (DNA chips) 899 (osteoarthritis) 905–906, immunohistochemistry 242– 143–144 desmoplastic small round cell 907 244, 245 DNA sequencing tumor 880 denaturing gradient gel multiple desmoids 240 next-generation (NGS) 123, Ewing sarcomas/PNETs electrophoresis (DGGE) site of occurrence 238 144–145 atypical form 860–861, 861 142 abdominal wall 239–241 pyrosequencing 144 PNET 861, 861 denaturing high-performance extra-abdominal 36,239–241 Sanger (dideoxy) method 144 typical form 860, 860 liquid chromatography intra-abdominal 240–241, docetaxel 1030–1031 granular cell tumor 673 (DHPLC) 142 245, 487 Doppler ultrasound 14 hemangioma 555 dendritic reticulum cell sarcoma terminology 233 double minutes (dmins) 130 Langerhans cell histiocytosis 965–968, 967 treatment 241 neuroblastoma 875, 877 961 dermatofibroma see benign hormonal 1032 doxorubicin 1017, 1029 leiomyosarcoma 455 fibrous histiocytoma radiotherapy (adjuvant) combination therapies 1029– malignant fibrous dermatofibroma with monster 1031 1030 histiocytoma cells (atypical fibrous surgery 239–241, 1015 high-dose therapy 1030 myxoid 357–358 histiocytoma) 226–228, TKIs 241–242, 1032 DSRCT see desmoplastic small pleomorphic 362 227 desmoplastic fibroblastoma round cell tumor Merkel cell carcinoma 801 dermatofibrosarcoma 208–209 duodenal gangliocytic mesothelioma 789, 790 protuberans (DFSP) desmoplastic melanoma 702–704 paraganglioma 737–738, MPNST 680 336–346 histopathology 702–703, 703 739 neuroblastoma 874, 875 appearance 338, 338 immunohistochemistry 704, duodenal GIST see small perineurioma 668, 671 Bednar tumor (pigmented 704 intestinal GISTs pheochromocytoma 728 variant) 342 desmoplastic mesothelioma Dupuytren’s contracture see rhabdoid tumor 883, 883 clinical features 336–338, 342 788–789, 790, 788 palmar fibromatosis rhabdomyoma 529

1041

Index

electron microscopy (cont.) collagenous fibroma 208 myxoinflammatory epithelioid fibrous histiocytoma schwannoma 655 dermatofibrosarcoma fibroblastic sarcoma 354 225, 226 melanotic 663 protuberans 337, 342 myxoma epithelioid gastric GISTs 479, EMA see epithelial membrane desmoids 238–240 cardiac 313 480 antigen elastofibroma 204 cutaneous 306 epithelioid embolization of tumors 13 epithelioid sarcoma 754 intramuscular 299 hemangioendothelioma embryonal rhabdomyosarcoma Ewing sarcomas/PNETS 857 juxtaarticular 302 (EHE) 595–599 (ERMS) 532, 533–535 extremity sarcoma 1014 nerve sheath 665 cytology 997, 998 appearance 535 fasciitis neuroblastoma 870 differential diagnosis 570, 997 botryoid 533–535 cranial 249 neurofibroma 645, 650 hepatic 596–597 cytology 993–994, 994 intravascular 185 neuroma, palisaded histopathology 596–597, genetics 108, 535 ischemic 195 encapsulated 642 597–598 histopathology 534–535, 536 necrotizing 198 neurothekeoma 277 hepatic 597, 598 botryoid subtype 535, 537 nodular 182 nuchal-type fibroma 207 pulmonary 597, 599 immunohistochemistry 51, 53, proliferative 191 oral giant cell fibroma 213 sarcoma-like 547 fibromyxoid sarcoma 346 oral irritation fibroma 212–213 (pseudomyogenic) 601 mixed with alveolar form 544 Gardner fibroma 262 ossifying fibromyxoid tumor immunohistochemistry 47, EMC see extraskeletal myxoid giant cell angiofibroma 330 317 597–598, 600 chondrosarcoma giant cell fibroblastoma 342 palmar fibromatosis 233 pulmonary 596–597 emperipolesis 957 GISTs 474, 482 paraganglioma 726, 728, 735, sarcoma-like endometrial carcinoma see glomus tumor 625 737 (pseudomyogenic) malignant mixed granular cell tumor 672 penile fibromatosis 237 599–601 müllerian tumor hemangioendothelioma 596 pheochromocytoma 726 epithelioid hemangioma 104, endometrial stromal sarcoma hemangioma plantar fibromatosis 233 554, 569–570 (ESS) 501–503 epithelioid 569 retroperitoneal fibrosis 199 appearance 570 differential diagnosis 464 hobnail 565 rhabdomyoma 528 histopathology 570, 571–572 genetics 123, 503 infantile 554 rhabdomyosarcoma 532 epithelioid histiocytoma, solitary histopathology 501, 502 intramuscular 563 schwannoma 654, 656 (reticulohistiocytoma) hormonal therapy 1031 lobular capillary 557 solitary fibrous tumor 324, 330 962–964, 964 immunohistochemistry 70, spindle cell 572 superficial angiomyxoma 306 epithelioid inflammatory 502, 503 hibernoma 398–399 synovial sarcoma 744 myofibroblastic tumor endometriosis 506–507 juvenile xanthogranuloma tendon sheath fibroma 209 (epithelioid histopathology 507, 508 961 tenosynovial giant cell tumor myofibroblastic sarcoma) endothelial biology 553–554 Kaposi’s sarcoma 612–613 915, 918, 920 290, 291 endothelial markers 41–48 leiomyoma 446, 450 truncal sarcoma 1014 epithelioid leiomyoma/ Enneking surgical staging system leiomyosarcoma 456, 457, 457, epidermal inclusion cyst, leiomyosarcoma 463, 463 6, 18 466 ruptured 933, 933 epithelioid mesothelioma 785, eosinophilic fasciitis 195–197, 198 lipoblastoma 405 epididymal tumors 786 ependymoma lipoma 379, 388, 392–393 adenomatoid tumor 777 epithelioid nerve sheath tumor abdominal 711–713 liposarcoma melanotic neuroectodermal (benign) 664, 665 cytology 991–992, 992 dedifferentiated 425 tumor of infancy 709 epithelioid rhabdomyosarcoma histopathology myxoid 430 epigenetics (epimutations) 116, 544–545, 545 abdominal 713 pleomorphic 436 136 epithelioid sarcoma 753–758 myxopapillary 711, 712 well-differentiated 417 SDH-deficient GIST 483 appearance 755 immunohistochemistry lymphangioma 582 SDH-deficient paraganglioma clinical features 754–755 712–713 lymphangiomyoma(tosis) 518 725 cytology 1003–1004, 1004 myxopapillary 711, 740, 991– lymphedema in the obese small round blue cell tumors differential diagnosis 756–758, 992 patient 410 856 1004 epidemiology 2–3, 11, 975 malignant fibrous as a therapeutic target 1032 genetics 758 aggressive angiomyxoma 498 histiocytoma epithelial cell markers see histopathology 755, 756–757 alveolar soft part sarcoma 895 inflammatory 367 epithelial membrane proximal-type 758, 759 angioleiomyoma 446 myxoid antigen; keratins immunohistochemistry 47, 74, angiolipoma 388 (myxofibrosarcoma) 357 epithelial membrane antigen 755, 757, 758 angiomatoid fibrous pleomorphic 362 (EMA) 69 proximal-type 758 histiocytoma 283 melanoma 696, 702 perineurioma 668, 671, 671 epithelioid sarcoma-like angiomyolipoma 513 meningioma, ectopic 714 rhabdoid tumor 884 hemangioendothelioma angiosarcoma 601, 602 Merkel cell carcinoma 798 epithelial tumors of unknown 599–601, 601 atypical fibroxanthoma 364 mesodermal stromal polyps histogenesis see epithelioid schwannoma 657, benign fibrous histiocytoma 491 epithelioid sarcoma; 658, 661 220 mesothelioma 782–783 synovial sarcoma Epstein–Barr virus (EBV) calcifying aponeurotic fibroma MPNST 678, 683–684 epithelioid angiomyolipoma 517, Burkitt lymphoma 949 257 myofibroblastoma 517 diffuse large B-cell lymphoma cellular blue nevus 694 breast 218 epithelioid angiosarcoma 608, 947 clear cell sarcoma of tendons palisaded 214 609–610 leiomyosarcoma 3–4, 467–468, 705 myofibroma 267 cytology 997, 998 468

1042

Index

ER-positive tumors see estrogen typical form 860, 860 extranodal marginal zone fat necrosis (lipogranuloma) 407– receptor (ER)-positive genetics 104, 856 lymphoma 946–947, 947 409, 408, 977–978, 978 tumors BCOR-CCNB3 fusion 866 extrarenal rhabdoid tumor see FDG-PET (positron emission Erdheim–Chester disease CIC-DUX4 fusion 865–866 rhabdoid tumor, extrarenal tomography) 12 957–960, 959 EWSR1/ETS family fusions extraskeletal chondroma see female genital tract tumors 491 ERG/ERG (ETS-related gene) 107–108, 123, 125–126, chondroma of soft parts see also ovarian tumors; as an IHC marker 47–48, 610 856, 863–864 extraskeletal mesenchymal uterine tumors; vaginal fusion with EWSR1 864 EWSR1/non-ETS family chondrosarcoma tumors; vulvar tumors fusion with FUS 127, 864 fusions 864–865 (MCHSA) 832–834 differential diagnosis 464, eribulin 1030 FISH probes 97, 864 appearance 833 492–493, 497, 500–503 ERMS see embryonal t(11;22) (q24;q12) cytology 1000, 1000 ferruginous bodies 782–783, 783 rhabdomyosarcoma translocation 106, 863 histopathology 832–833, 833 FET proteins 125 see also EWSR1 eruptive xanthoma 932 histopathology 858, 858 imaging 832, 833 fusions/translocations; erythema migrans 904 atypical form 858–859, 859 immunohistochemistry 57, FUS gene fusion esophageal tumors BCOR-CCNB3 fusion 833–834, 834 fetal rhabdomyoma 528–529 GISTs 475–476, 477 sarcoma 866, 866–867 extraskeletal myxoid histopathology 528, 528 intramural leiomyoma CIC-DUX4 fusion sarcoma chondrosarcoma (EMC) FFCC (French Federation of 449–451, 451, 451 866 830–832 Cancer Centers) grading leiomyomatosis 450 PNET 860, 860 appearance 830 system 4–5 ESS see endometrial stromal typical form 858, 859 cytology 1006, 1007 FH/fumarate hydratase 138 sarcoma history 856–857 genetics 126, 832 fumarase-deficient leiomyoma estrogen receptor (ER)-positive imaging 857, 858 histopathology 830, 831–832 461, 463 tumors 72–73 immunohistochemistry 71–72, hybrid with synovial sarcoma fibroblast biology 181–182 desmoids 1032 861–863, 866 750 fibroblast growth factors (FGFs) leiomyoma 72–73 CD99 71, 861–862, 862, 865 imaging 830, 830 182, 834, 837 abdominal 73, 459–460, 459 chromogranin 54 immunohistochemistry 54, 57, fibroblastic proliferations 181, benign metastasizing FLI1 48, 862, 862 831 249, 277, 336 see also (pulmonary nodules) keratins 66, 862 extraskeletal osteosarcoma see individual lesions 464–465, 465 synaptophysin 53, 862 osteosarcoma, fibroblastic reticulum cells uterine 459–463, 461–462 prognosis 864–865 extraskeletal normal 968 vulvar 459 Ewing-like sarcoma, extremity angiosarcoma (Stewart– sarcoma 968 and response to hormonal undifferentiated 865 Treves syndrome) 4, 603– fibroblastic sarcoma see acral therapy 1031 EWSR1 gene fusions/ 604, 604 myxoinflammatory etiology 3–4 translocations (EWS eye see orbital tumors fibroblastic sarcoma angiosarcoma 3–4, 593 RNA-binding protein 1) eyelids, xanthelasma 932 fibrocartilaginous pseudotumor, atypical fibroxanthoma 364 104, 124, 126 nuchal 207–208, 208 EBV-associated tumors 3–4, ATF1-CREB1 126, 287–288, Fabry disease 560 fibrodysplasia ossificans 467–468, 947, 949 707–708 Factor XIIIa 62 progressiva 845 Kaposi’s sarcoma 3, 612 DDIT3 126–127 falciform ligament PEComa 521 imaging 845, 846 Merkel cell carcinoma 798, ETS genes (FLI1, ERG) 107–108, familial adenomatous polyposis fibrohistiocytic tumor see 801–802 123, 125–126, 863–864 (FAP) 137–138, 238, 240, plexiform fibrohistiocytic mesothelioma 782–783 activity of the fusion 245–246, 263 tumor ETS gene/transcription factor products 136, 856 familial cancer syndromes 117– fibroids (uterine leiomyoma) family 47–48, 125–127, and prognosis 864 119 see also individual 459–463 863–864 see also ERG/ FISH probes 97, 864 syndromes histopathology 461–462, 461 ERG; ETV6; FLI1 NFATC2 865 familial cylindromatosis 763 fibrolipoma 382 ETV6 (ETS variant gene 6), NR4A3 126, 832 familial GIST syndrome 486–487 neural 385–386, 386–387 fusion with NTRK3 124, PATZ1 865 familial glomuvenous fibrolipomatous hamartoma of 127–128, 294 PBX1 126, 760–761 malformation 630–631 nerve 385–386, 386–387 everolimus 133 POU5F1 126, 760–761, 865 familial infiltrative fibromatosis fibroma see also Ewing sarcoma family of tumors SMARCA5 865 137 myofibroblastoma; 856–867 SP3 865 fascicular sign 30 neurofibroma adamantinoma-like 99 WT1 126–156, 881 fasciitis 181 calcifying aponeurotic 256–257 appearance 857–858, 858 excisional biopsy 1013 cranial 182, 249–251, 250 collagenous 208–209, 210–211 BCOR-CCNB3 sarcoma 866– external beam radiation therapy eosinophilic 195–197, 198 dermatomyofibroma 212, 215 867 (EBRT) 1015–1016 intravascular 182, 185–189, 190 elastofibroma 22, 34, 204–206, chemotherapy 1018, 1031 extra-abdominal desmoids 36, ischemic 195, 197 205–206 CIC-DUX4 sarcoma 865–866 239–241 necrotizing 197–199, 198–199 Gardner fibroma 207, 262, clinical features 857, 1004 extragastrointestinal nodular see nodular fasciitis 262–263 cytology 1004–1005, 1005 gastrointestinal stromal proliferative 190–194, 193– infantile digital fibroma/ differential diagnosis 753, 865, tumors (EGISTs) 481–482 194, 982 fibromatosis 254–256, 1004–1005 extramedullary myeloid tumor fasciitis ossificans (panniculitis 256–257 electron microscopy see myeloid tumor, ossificans) 182–183 nuchal-type 206–207, 207 atypical form 860–861, 861 extramedullary (myeloid fat atrophy (lipodystrophy) 407, oral PNET 861, 861 sarcoma) 408 giant cell 213, 218

1043

Index

fibroma (cont.) histopathology 293–294, focal cutaneous mucinosis 310, 311 with DDIT3 126–127, 434–435 irritation 212–213, 217 293–294 focal myositis 194, 531, 531 with FLI1 or ERG 127, 864 skin inflammatory 288 focal tumoral calcium fusion genes 103, 106–107, pleomorphic 211–212, 215 sclerosing epithelioid 74, 336, pyrophosphate dihydrate 117–129 sclerotic 211, 214 350–351, 351 deposition disease ALK 124, 127, 292, 953–954 tendon sheath 209–211, 213–214 fibrosarcoma-like lipomatous (tophaceous pseudogout) ASPSCR1-TFE3 129, 900 fibromatosis see also desmoid neoplasm 423 838–839 BCOR 124, 866 fibromatosis (desmoid fibrosarcomatous transformation histopathology 839, 841 CIC-DUX4 865 tumor); infantile of DFSP 341–342, 341 imaging 839, 840 COL1A1-PDFGB 128–129, 345 myofibroma/ fibrous dysplasia of bone follicular dendritic (reticulum) cell ETV6-NTRK3 124, 127–128, myofibromatosis; 300, 301–302 sarcoma 965–968, 967 294 neurofibromatosis type 1; fibrous hamartoma of infancy follicular lymphoid hyperplasia EWSR1 see EWSR1 gene neurofibromatosis type 2 265–266, 265–266 941, 944 fusions/translocations familial infiltrative 137 fibrous histiocytoma of tendon follicular lymphoma 945–946, 946 FUS 124, 864 gingival 253, 253–254 sheath see tenosynovial foot see also acral FUS-CREB3L1/FUS- imaging 34–35, 36 giant cell tumor myxoinflammatory CREB3L2 127, 348–349, infantile diffuse fibromatosis fibrous mesothelioma see solitary fibroblastic sarcoma; 351 260–261, 261 fibrous tumor plantar fibromatosis FUS-DDIT3 126–127, juvenile hyaline 253, 269–272, fibrous umbilical polyp 251–253, hemangioma 562 434–435 271–272 252 infantile digital fibroma HMGA2 124, 382 lipofibromatosis 257–260, fibroxanthoma see atypical 254–256, 256–257 JAZF1-SUZ12 123, 503 260–261, 283 fibroxanthoma; Morton neuroma 30, 31, 638, NAB2-STAT6 330 palmar/plantar 233–237 tenosynovial giant cell 639 NCOA2 125, 834 palmar histopathology 234– tumor pleomorphic hyalinizing PAX-FOXO1A (FKHR) 129, 235, 235 FICTION technique 99 angiectatic tumor 535, 539–540, 542, 547 plantar histopathology 235– filariasis 603 369–372, 371, 371 PHF1 125, 318 236, 236–237 fine-needle aspiration (FNA) Proteus syndrome 272 PLAG1 125, 407 penile 237–238, 238 975, 1012 Fordyce angiokeratoma 560 ROS1 125, 127 terminology 233, 249, 260– FISH see fluorescence in situ foreign body reactions 3 SS18-SSX 123, 128, 136, 753 261, 266 hybridization angiosarcoma 607–608 TFE3 125, 129, 599, 900 fibromatosis colli 251 fixatives 8 cotton fiber granuloma 934, TFK 126 appearance 251 in FISH 98 935 TMPRSS2 125 histopathology 251, 252 recovery of DNA/RNA from PVP accumulation 933–934, USP6 (TRE17) 125 fibromyxoid sarcoma (low- fixed specimens 140–141 933–934 WWTR-CAMTA1 598–599 grade) (LGFMS) 346–349 FKHR (FOXO1A)-PAX gene to silicone YAP1-TFE3 599 see also sclerosing fusion 129 ALCL 953 epithelioid fibrosarcoma FLI1/FLI1 (Freund’s leukemia granuloma 409, 410 gadolinium contrast agent 13–14 appearance 347 integration site) synovial 911, 912 gadolinium-associated fibrosis clinical features 346 as an IHC marker 48, 611, 862, synovial 936, 936 cytology 986–987, 986 862 to bone cement 911, 911 gallium scans 11–12 differential diagnosis 347–348, fusion with EWSR1 107–108, to carbon fibers/graphite gangliocytic (duodenal) 987 125, 863–864 911–912, 912 paraganglioma 737–738, genetics 101, 348–349 activity of fusion products to metal fragments 912–913, 739 histopathology 346–347, 136, 856 913 ganglion cyst 32, 303–305 347–348 and prognosis 864 to polyethylene wear histopathology 304–305, giant hyaline rosette variant florid reactive periostitis of the particles 910, 911 304–305 350 tubular bones of hands to silicone 911, 912 imaging 33 higher-grade variant 349 and feet (fibro-osseous formalin fixatives 8 ganglion of distal interphalangeal hyalinizing spindle cell tumor pseudotumor of the Fournier’s gangrene 198 joint 305–306, 306 with giant rosettes 347 digits) 845–847, 847 FOXL2 mutations 506 ganglioneuroblastoma 868–869, immunohistochemistry 74, 347 flow cytometry 976 FOXO1A (FKHR)-PAX gene 869–870, 872, 874 fibromyxoma see superficial acral fluid collections 32–33 fusion 129, 535, 539–540, ganglioneuroma 872 fibromyxoma fluorescence in situ hybridization 542, 547 appearance 872 fibro-osseous pseudotumor of (FISH) 95 French Federation of Cancer gastrointestinal/mucosal 639 the digits 845–847, 847 in Ewing sarcomas 97, 864 Centers (FFCC) grading histopathology 868, 869, 872, fibrosarcoma see also MYCN amplification 98–99, system 4–5 872 dermatofibrosarcoma 877 frozen tissue specimens 8 mucosal 641 protuberans; probes 97–99, 99–100, 119 fumarate hydratase (FH) 138 immunohistochemistry 55 myofibrosarcoma; pros and cons 102 fumarase-deficient leiomyoma with MPNST/ myxofibrosarcoma specimen preparation 98, 140– 461, 463 pheochromocytoma 686, adult 336, 351–352 141, 976 fungal synovitis 908–909 732 cytology 984, 985 specimen selection 95–97 FUS (TLS) gene fusions 124, ganglioneuromatosis 639 spindle cells 352–353 technical variations 99–100 126–127 Gardner fibroma 262–263 infantile 292–294 FNA (fine-needle aspiration) with CREB3L1 or CREB3L2 differential diagnosis 207, 263 appearance 293 975, 1012 127, 348–349, 351 histopathology 262, 262

1044

Index

immunohistochemistry 262,262 genetics aggressive angiomyxoma 501 epithelioid sarcoma 758 Gardner syndrome KIT and PDGFRA alveolar soft part sarcoma 129, Erdheim–Chester disease (adenomatous polyposis mutations 131–132, 475, 900 958–960 with desmoids) 137–138, 486–487, 1024–1026 analytical techniques 92 Ewing sarcomas/PNETs see 238, 240, 245–246 other 108, 487 cDNA microarrays 143–144 Ewing sarcoma family of gastric GIST 476–479 SDH-deficient form 483 CGH/aCGH 100–101, 123, tumors, genetics appearance 477, 477–478 histopathology 475 143 fasciitis, nodular 185 genetics esophageal 477 clonality assay (HUMARA) fibrodysplasia ossificans KIT and PDGFRA gastric 478–479, 478–480 138–139 progressiva 845 mutations 486–487 in NF1 patients 484 conventional cytogenetics 8, fibromyxoid sarcoma 101, succinate dehydrogenase- post-treatment 488 92–95 348–349 deficient 482–483 rectal 481, 482 for detection of gene follicular lymphoma 946 histopathology 478–479 SDH-deficient tumors 483, amplification 98–99, 130 gingival fibromatosis 253 epithelioid type 479, 480 483 DNA sequencing 123, 144– GISTs hypercellular type 479, 479 small intestinal 479–480, 481 145 KIT and PDGFRA palisaded-vacuolated type imaging of “tumor within a FISH (and variants) 95–100, mutations 131–132, 475, 478, 479 tumor” 1025 102, 119 486–487, 1024–1026 sarcomatous type 479, 479 immunohistochemistry 70, PCR (and variants) 119– other 108, 487 sclerosing spindle cell type 484–486, 485 123, 141–143, 977 SDH-deficient form 483 478, 478 CD34 47, 485–486 specimen preparation and glomangiopericytoma 631 origin of omental GISTS 482 desmin 50, 486 handling 8, 92–93, 95–97, glomus tumor 630–631 gastric glomus tumor 624–626 heavy caldesmon 52, 486 140–141 granulosa cell tumor 506 gastrointestinal autonomic nerve keratins 66, 486 anaplastic large cell lymphoma growth factor signaling tumor (GANT) see KIT 74, 484, 485 952–954 pathways 136–138 gastrointestinal stromal S100 57 aneurysmal bone cyst 104, hemangioendothelioma 598– tumor (GIST) SDH-deficient tumors 483 844–845 599, 601 gastrointestinal carcinoma location 474–475 angioleiomyoma 447 hemangioma 104, 575, 587 808–809 appendix 481 angiomatoid fibrous hemangiopericytoma 330, 333 gastrointestinal clear cell colon 480–481 histiocytoma 287–288 hereditary cancer syndromes sarcoma 708, 709 esophagus 475–476 angiomyolipoma 517–518 117–119 see also gastrointestinal leiomyoma extragastrointestinal 481–482 angiosarcoma 611 individual syndromes 448–449 rectum 481 atypical fibroxanthoma 366 infantile fibrosarcoma 294 appearance (esophageal) 450, small intestines/duodenum benign fibrous histiocytoma inflammatory myofibroblastic 451 476, 479, 483–484 228 tumor 107, 127, 292 intramural 449–451, 451 stomach 476–479 Burkitt lymphoma 949 juvenile hyaline fibromatosis muscularis mucosae 451–452, in NF1 patients 483–484, 653 Carney complex 309–310 271–272 452 succinate dehydrogenase- cell cycle regulatory pathways Langerhans cell histiocytosis gastrointestinal leiomyosarcoma deficient 482–483, 488, 134–136 961 457 1025 cellular angiofibroma 497 leiomyoma 447, 450–451, 462– gastrointestinal lipoma 385 surgery 487, 1020 chondroma 827 463 gastrointestinal mucosal palliative 1026 chromosomal abnormalities leiomyosarcoma 455 (ganglio)neuroma 639 targeted therapies amplification 98–99, 101, lipoblastoma 407 gastrointestinal nerve sheath future directions 1027 130–131, 611, 875–876 lipoma 131, 382 tumor 661, 662 SDH-deficient tumors 488, fusion see fusion genes angiolipoma 390 gastrointestinal 1025 hyperdiploidy 548, 876–877 chondroid 404 neurofibromatosis 653 TKIs, adjuvant 1020, 1026– multiple/complex 103, 108– hibernoma 402 gastrointestinal paraganglioma 1027 110, 123, 134 lipomatosis 387 (duodenal gangliocytic) TKIs, first line 132, 487–488, translocations 103–108, 117, spindle cell/pleomorphic 737–738, 739 1024–1026 120 394 gastrointestinal PEComa 521, TKIs, resistance 132, 486, trisomy 237, 246, 294, 506, liposarcoma 101 522 1020, 1024–1025, 1025, 921 dedifferentiated 108, 430 gastrointestinal schwannoma 1027 clear cell sarcoma 707–708 myxoid 106, 126–127, 434– 487, 660–661 terminology 474 collagenous fibroma 209 435 appearance 660, 660 gastrointestinal synovial sarcoma cylindroma 763 pleomorphic 437–438 histopathology 660–661, 661 746 dermatofibrosarcoma well-differentiated 102, 131, gastrointestinal stromal tumor gastrointestinal vascular protuberans 128–129, 345 416–423 (GIST) 474 proliferation related to desmoid fibromatosis malignant fibrous appearance 477, 477–478, intestinal intussusception APC 137–138, 245–246 histiocytoma 134, 359, 480 580, 581 CTNNB1 138, 246 362–364 clinical features 474–476, GCT see granular cell tumor other 240, 246 malignant mixed müllerian 479, 481–483 gel electrophoresis 142 desmoplastic small round cell tumor 505 differential diagnosis 292, 457, gemcitabine 1030–1031 tumor 126–156, 881 mastocytosis 956 481–482, 487 gene expression profiling 123, elastofibroma 206 melanoma 702, 704 familial syndromes 482–483, 143–144, 977 endometrial stromal sarcoma melanotic neuroectodermal 486–487 genetics 115 see also epigenetics 123, 503 tumor of infancy 709

1045

Index

genetics (cont.) tenosynovial giant cell tumor differential diagnosis 628–629 scalp 603 mesenchymal 105, 914, 920–921 genetics 630–631 atypical fibroxanthoma 365 chondrosarcoma tumoral calcinosis 838 histopathology 625–626, 627– benign fibrous histiocytoma (extraskeletal) 834 vascular malformations 587 628 220–221, 221 mesothelioma 794 germ cell tumors atypical/malignant 630 aneurysmal variant 224 miRNA 116–117, 129, 136 with angiosarcoma 607 glomangioma 628 cellular variant 225 mixed tumor 760–761 dysgerminoma 818 glomangiomyoma 629 chondroma 826, 826 MPNST 108–110, 117, 682 seminoma 48, 817–818, 818 glomus bodies 624 chordoma 766 myxofibrosarcoma 359 GFAP (glial fibrillary acidic intramuscular 629 collagenous fibroma 208, 210 myxoid chondrosarcoma protein) 73–74, 712–713, immunohistochemistry 626– dermatofibrosarcoma (extraskeletal) 126, 832 712 627, 631 protuberans 338, 338 myxoinflammatory giant cell angiofibroma 330, 331 glycogen desmoids 242, 242 fibroblastic sarcoma 355– giant cell fibroblastoma 342–343, in alveolar soft part sarcoma elastofibroma 204–205, 205 356 343 897, 898 epithelioid sarcoma 755 myxoma 301–303, 314–315 giant cells 344 in Ewing sarcoma cells 859– Ewing sarcomas/PNETs neuroblastoma 130–131, 875– giant cell fibroma of the oral 860 857–858, 858 877 cavity 213, 218 Gorlin syndrome (nevoid basal fasciitis MYCN amplification 99, giant cell hyaline angiopathy cell carcinoma intravascular 186–187, 190 130, 875–876, 877 (pulse granuloma) 929, syndrome) 529 necrotizing 198, 198 and prognosis 876–878 929 gossypiboma (cotton fiber nodular 183, 185 neurofibromatosis type 1 giant cell malignant fibrous granuloma) 934, 935 proliferative 192–200, 193 (NF1) 108–110, 132–133, histiocytoma (MFH) gout 841, 903, 907, 909–910 see fibromatosis colli 251 637, 653 368–369, 989 also pseudogout fibromyxoid sarcoma 347 neurofibromatosis type 2 cytology 989 grading of tumors 4–5, 75–76 gingival fibromatosis 253 (NF2) 133, 637 histopathology 369, 370 granular cell fibrous GISTs 477, 477–478, 480 nomenclature giant cell tumor of soft parts histiocytoma 228 glomus tumor 625, 626 cytogenetics 94–96 282–283, 367–368, 369 granular cell tumor (GCT) 672– granular cell tumor 673, 675 of individual genes 146–153 giant cell tumor of tendon sheath 674 granulosa cell tumor 506 ossifying fibromyxoid tumor see tenosynovial giant cell appearance 673, 675 hemangioendothelioma 318 tumor cytology 1002, 1002 epithelioid 596, 597 osteosarcoma 849 Giemsa stain 94 differential diagnosis 673–674 PILA 593 palmar/plantar fibromatosis 237 gingival fibromatosis 253 histopathology 672–673, 673, hemangioma 559, 564, 570, paraganglioma 724–726 appearance 253 675 573 PEComa 513, 517–518, 523 histopathology 253, 254 malignant 676 hemangiopericytoma 331 perineurioma 669, 671 gingival granular cell tumor pseudoepitheliomatous hibernoma 399, 400 phosphaturic mesenchymal (gingival epulis of hyperplasia 674 infantile digital fibroma 254– tumor 837 newborn) 676, 677 immunohistochemistry 56–57, 255, 256 plexiform fibrohistiocytic histopathology 676, 677 673, 675 infantile fibrosarcoma 293 tumor 283 immunohistochemistry 676, malignant 675–676 inflammatory myofibroblastic proliferative myositis 194–195 677 granular cell tumor (GCT), tumor 289, 289 Proteus syndrome 272–273 GIST see gastrointestinal stromal gingival 676, 677 juvenile hyaline fibromatosis rhabdoid tumor 133, 882, 885– tumor histopathology 676, 677 271 886 Gleevec see imatinib mesylate immunohistochemistry 676, 677 juvenile nasopharyngeal rhabdomyoma 529–530 glial fibrillary acidic protein granuloma annulare, deep 927– angiofibroma 263–264, rhabdomyosarcoma 548 (GFAP) 73–74, 712–713, 929, 928 263 alveolar 129, 134–135, 539– 712 granulomatous synovitis 907–909 juvenile xanthogranuloma 541, 542, 878–879 glioma, nasal 710 mycobacterial 907–908, 909 962 embryonal 108, 535 gliomatosis 710–711, 711 in sarcoidosis 907 Kaposi’s sarcoma 613 epithelioid 545 glomangioma 628 granulosa cell tumor 505–506 leiomyoma mixed alveolar and glomangiomyoma 629 appearance 506 esophageal 450, 451 embryonal 544 glomangiopericytoma 631–632, histopathology 505–506, 506 retroperitoneal 459 pleomorphic 542 632 immunohistochemistry 506, leiomyomatosis, peritoneal and prognosis 547 glomangiosarcoma 626, 630 507 465, 466 spindle cell 538 glomeruloid hemangioma 564– gross anatomy/morphology 7 leiomyosarcoma 452–453, 453 schwannoma 660–661 565, 566 alveolar soft part sarcoma 896, uterine 467 sclerosing epithelioid glomulin 630–631 896 vena cava 458 fibrosarcoma 351 glomus bodies 624, 624 angiomatoid fibrous lipoblastoma 406 solitary fibrous tumor 330 glomus jugulare tumor histiocytoma 284–285 lipoma 379, 380 superficial angiomyxoma 309– (jugulotympanic angiomyofibroblastoma-like chondroid 402 310 paraganglioma) 737, 738 tumor (male) 497 lipoma arborescens 383 synovial chondromatosis 830 glomus tumor 624–631 angiomyolipoma 514 myelolipoma 391 synovial sarcoma 123, 128, 753 appearance 625, 626 angiosarcoma neural fibrolipoma 386 TCA cycle dysfunction 138, atypical/malignant 626 breast 605 spindle cell lipoma 394 724–725 clinical features 624–625 deep soft tissue 606 thymolipoma 385 tendon sheath fibroma 211 cytology 998, 999 lymphedematous arm 604 liposarcoma

1046

Index

dedifferentiated 425, 426 superficial angiomyxoma 306– infantile digital fibroma 254– congenital forms 556–557 well-differentiated 419, 419 307 256, 256–257 differential diagnosis 381–382, lymphangioma 584 synovial chondromatosis 827– lipomatosis of the nerve 385– 557–558, 570 lymphangiomyoma(tosis) 828, 828 386, 386–387 epithelioid 104, 554, 569–570 518–519, 518 synovial sarcoma 746, 746 pacinian neuroma 640–641, genetics 104, 575, 587 malignant mixed müllerian tendon sheath fibroma 213 642 glomeruloid 564–565 tumor 504, 504 tenosynovial giant cell tumor sclerotic lipoma 395–396, 398 histopathology Merkel cell carcinoma 799, 799 diffuse intra-articular 919, Hand–Schüller–Christian disease acquired elastotic 577 mesenchymal 919 960 acquired tufted angioma chondrosarcoma localized 916, 916 Hashimoto–Pritzker disease 960 567, 568 (extraskeletal) 833 tumoral calcinosis 838, 838 heart see entries at cardiac angiomatosis 563–564, 565 mesothelioma, malignant 783 growth factors/growth factor heavy caldesmon (HCD) 50–51, arteriovenous 562 peritoneal 784 receptors 136 486 cavernous 559, 560 pleural 784 FGF 182, 834, 837 hedgehog signaling pathway 529 cherry 576 MPNST 678, 679, 684 HGF/MET 130, 136–137 hemangioendothelioma epithelioid 570, 571–572 multicystic peritoneal IGF/IGF1R 136–137 appearance glomeruloid 565, 566 mesothelioma 779 PDGFA receptor 132, 475, epithelioid 596, 597 hobnail 565–566, 567 myxofibrosarcoma 358 1024 PILA 593 infantile (juvenile capillary) myxoid chondrosarcoma PDGFB/PDGFRB 128–129, cytology (epithelioid) 997, 998 555, 556 (extraskeletal) 830 242 differential diagnosis 570, 997 intramuscular 562, 564 myxoinflammatory as therapeutic targets 137 epithelioid 570, 595–599, 997 intravascular pyogenic fibroblastic sarcoma 354 VEGF/VEGFR 47, 553, 555, epithelioid sarcoma-like 599– granuloma 557, 559 myxoma 593–594 601 lobular capillary 557–558, cardiac 314 Gs alpha mutations 301–302 genetics 598–599, 601 558 intramuscular 300, 300 gynecological tumors 491 see also hepatic 596–597 microvenular 576 neuroblastoma 871, 872 ovarian tumors; uterine histopathology spindle cell 573–575, 573– ganglioneuroma 872 tumors; vaginal tumors; epithelioid 596–597, 597– 574 neurofibroma vulvar tumors 598 venous 563 cutaneous 653 differential diagnosis 464, epithelioid sarcoma-like verrucous 560–561, 561 intraneural 648 492–493, 497, 500–503 601, 601 hobnail 565–566 plexiform 649 hepatic 597, 598 imaging 25–27 neurofibromatosis type 1 (café HAART (highly active kaposiform 567–568, 568– angiomatosis 27, 29 au lait spots) 653 antiretroviral therapy) 569 capillary 26, 28 organizing hematoma 926, 926 407 PILA 593, 594 cavernous 25–26, 26–27 ossifying fibromyxoid tumor hamartin (TSC2) 517–518 pulmonary 597, 599 immunohistochemistry 555, 319 hamartoma retiform 594–595, 595 566, 570, 572, 574 palisaded myofibroblastoma angiomyomatous hamartoma imaging 27 infantile 554–555 218 of lymph node 444, 445 immunohistochemistry intramuscular 381–382, 562 palmar fibromatosis 233–234 fibrolipomatous hamartoma epithelioid 47, 597–598, 600 lobular capillary 557–558 papillary endothelial of nerve 385–386, epithelioid sarcoma-like microvenular 575 hyperplasia 577 386–387 601, 601 spindle cell 571–575 papillary intralymphatic fibrous hamartoma of infancy kaposiform 568 venous 562 angioendothelioma 593 265–266, 265–266 PILA 593–594, 594 verrucous 560–561 paraganglioma meningiothelial 714–715, 715 kaposiform 567–568 hemangiopericytoma (HPC) carotid body 735 neuromuscular 530, 530, 638, papillary intralymphatic 330–333 retroperitoneal 729 638 angioendothelioma clinical features 331, 333 PEComa (renal capsule) 520 rhabdomyomatous (PILA) 593–594 differential diagnosis 330, 333 penile fibromatosis 237 mesenchymal hamartoma pulmonary 596–597 genetics 330, 333 pheochromocytoma 726 528–530, 530 retiform 594–595 histopathology 331–332, 332 phosphaturic mesenchymal smooth muscle hamartoma of hemangioma 554–575 see also glomangiopericytoma 631, tumor 835 skin 443–444, 444–445 angiokeratoma; 632 plantar fibromatosis 233–234 hand see also acral kaposiform lipomatous 332 Proteus syndrome 272 myxoinflammatory hemangioendothelioma imaging 27, 30 renal cell carcinoma (cutaneous fibroblastic sarcoma; acquired elastotic 575 immunohistochemistry 333 metastases) 809 chondroma of soft parts; acquired tufted angioma 566– lipomatous 332–333 rhabdoid tumor 883, 883 palmar fibromatosis 567 not pericytic 330–331 rhabdomyoma, oral 529 calcifying aponeurotic fibroma angiomatosis 27, 562–564 sinonasal rhabdomyosarcoma 531–532, 256–257 appearance 559, 564, 570, 573 (glomangiopericytoma) 535 digital mucoid cyst 305–306, arteriovenous 26, 561–562 631–632 schwannoma 654–655, 654 306, 307 cavernous 25–26, 558–559 hematoma 926–927 gastrointestinal 660, 660 digital papillary cherry 575 appearance 926, 926 melanotic 662, 662 adenocarcinoma 764– classification 554 histopathology 926–927, 927 solitary fibrous tumor 326, 326 765, 765–766 compared with vascular imaging 33 superficial acral fibromyxoma fibro-osseous pseudotumor of malformations/ hematopoiesis, extramedullary 311 the digits 845–847, 847 telangiectasia 554 390

1047

Index

hematopoietic cell markers 942 deep granuloma annulare 927– hormonal therapy 1031–1032 lipoblastoma 19,21 hematopoietic progenitor cell 929, 928 HPC see hemangiopericytoma lipoma 15,20–21, 23 antigen see CD34 dendritic reticulum cell HPV16 (human papillomavirus liposarcoma 6, 17,22–25, hemophilia, synovial pathology sarcoma 965–968, 967 16) 803 23–25 906–907, 907–908 Erdheim–Chester disease 957– HRAS oncogene 115–116 lymphangioma 27, 28 hemosiderin 960, 959 human herpesvirus 8 (HHV8) 3, malignant fibrous in angiomatoid fibrous fibroblastic reticulum cell 136, 612 histiocytoma 16–20, 21 histiocytoma 284–285, sarcoma 968 human milk fat globule protein mesenchymal 287 interdigitating reticulum cell see epithelial membrane chondrosarcoma in benign fibrous histiocytoma sarcoma 58, 702, 968 antigen (extraskeletal) 832, 833 222 juvenile xanthogranuloma 63, human papillomavirus 16 myositis ossificans 12, 842, 843 in pigmented villonodular 224, 961–962, 962–963 (HPV16) 803 myxoid chondrosarcoma synovitis 21,34 Langerhans cell histiocytosis HUMARA clonality assay 138– (extraskeletal) 830, 830 hemosiderotic fibrolipomatous 960–961, 960–961 139 myxoma 300 tumor 404, 404–405 malakoplakia 930, 931 hyaline vascular Castleman neuroblastoma 871 hemosiderotic synovitis 906–907, malignant (histiocytic disease 941–943, 944 neurogenic tumors 27–31 907–908 sarcoma) 965, 966 hyalinizing spindle cell tumor Morton neuroma 30, 31 Henderson–Jones disease see multicentric with giant rosettes 347, MPNST/BPNST 11–12, 31, synovial chondromatosis reticulohistiocytosis 964, 350 32 hepatic angiomyolipoma 513– 964 hypercalcemia 837, 882 in NF1 22 514, 514 mycobacterial pseudotumor hypercellular spindle cell GISTs schwannoma 20,31 hepatic angiosarcoma 3–4, 606, 929–930, 930 479, 479 nonspecific appearance 35–36 606 pulse granuloma 929, 929 hypercholesterolemia, lesions osteosarcoma (extraskeletal) hepatic hemangioendothelioma PVP accumulation 933–934, associated with see 848, 848 596–597, 598 933–934 xanthoma phosphaturic mesenchymal hepatic PEComa (falciform reticulohistiocytoma 962–964, hyperdiploidy 548, 876–877 tumor 834, 835 ligament) 521 964 hyperphosphatemia 837–838 pros and cons of different hepatocellular carcinoma 809, 809 Rosai–Dorfman disease 58, hyperthermia therapy 1028 modalities 11–15 hepatocyte growth factor (HGF)/ 956–957, 958–959 hypoglycemia 324 PVNS 21,34 MET receptor 130, 136– ruptured epidermal inclusion rhabdomyosarcoma 535, 539 137 cyst 933, 933 iatrogenic gingival hyperplasia synovial chondromatosis 34, herbicides 3 xanthogranulomatous 253 35, 827, 828 hereditary cancer syndromes inflammation 930–932, iatrogenic Kaposi’s sarcoma 613 synovial sarcoma 18 117–119 see also 931 IDH1/IDH2 genes (isocitrate tophaceous pseudogout 839, individual syndromes histiocytosis X see Langerhans dehydrogenase) 138, 575 840 hereditary gingival fibromatosis cell histiocytosis idiopathic retroperitoneal tumoral calcinosis 838, 838 see gingival fibromatosis histogenesis 2, 527, 899–900 fibrosis see imatinib mesylate hereditary leiomyomatosis and histoid leprosy 930 retroperitoneal fibrosis for desmoids 241–242 renal cell cancer 445–446, histopathology see under ifosfamide 1017, 1029–1030 for DFSP 129, 345–346 461, 462–463 individual lesions high-dose therapy 1030 for GISTs 132 hernia sac lipoma 380–381, 385 HIV/AIDS IGF/IGF1R (insulin-like growth as adjuvant therapy 1020, herpes simplex virus 313 bacillary angiomatosis 578 factor/receptor) 136–137 1026–1027 heterotopic ossification see granuloma annulare 927 IgG4 sclerosing disease 201, 292 as primary therapy 487–488, myositis ossificans Kaposi’s sarcoma 612 IHC see immunohistochemistry 1024–1025 HHF-35 (actin antibody) 49, 546 lipodystrophy 407, 408 imaging 11–36, 1012 resistance 486–488, 1020, HHV8 virus (human herpesvirus mycobacterial pseudotumor angiomatosis 27, 29 1024–1025 8) 3, 136, 612 929–930, 930 angiosarcoma 27 immunocytochemistry see hibernoma 396–402 HMB45 monoclonal antibody calciphylaxis 841, 842 immunohistochemistry appearance 399, 400 58–59 chondroma 825, 826 immunoFISH 99 cytology 978–979, 980 HMGA2 (HMGIC) gene collagenous fibroma 210 immunohistochemistry (IHC) histopathology 399–400, 400– in aggressive angiomyxoma cranial fasciitis 250 41–77, 975 401 501 cystic masses 31–34 adenomatoid tumor 777 brown fat 398 fusions 124, 382 elastofibroma 22,34 adrenocortical carcinoma 60, imaging 14,21–22 in lipoma/lipomatosis 382, 387 Ewing sarcomas/PNETs 857, 815 hidradenoma (eccrine hobnail hemangioma 565–566, 858 alveolar soft part sarcoma acrospiroma) 762, 763 567 fibrodysplasia ossificans 898–899, 899 high-performance liquid Hodgkin’s disease 367, 953 progressiva 845, 846 anaplastic large cell lymphoma chromatography (HPLC) Hoffa disease 384 fibromatosis 34–35, 36 953–954 142 Homer Wright rosettes ganglion cyst 32, 33 angioleiomyoma 447 highly active antiretroviral neuroblastoma 867, 872, 874 GIST “tumor within a tumor” angiomatoid fibrous therapy (HAART) 407 PNETs 860, 860 1025 histiocytoma 285 histiocytic markers 58, 61–62 homogeneous staining regions hemangioma 25–27, 26–28 angiomyolipoma 59, 59, 60, 61, histiocytic proliferations (hsrs) 130 hemangiopericytoma 27, 30 517 crystal-storing histiocytosis neuroblastoma 875, 877 lipomatous lesions 19–25 angiosarcoma 45, 66, 608–610, 964–965, 965–966 homovanillic acid 870 hibernoma 14,21–22 611

1048

Index

atypical fibroxanthoma 366, giant cell angiofibroma 330 mastocytoma/mastocytosis 956 osteosarcoma 74–75, 849 367 GIST see gastrointestinal melanoma 56, 58–61, 60, 74, ovarian carcinoma 815–816 benign fibrous histiocytoma stromal tumor (GIST), 699–701, 700–701 palmar/plantar fibromatosis 222–225, 223, 227 immunohistochemistry desmoplastic 704, 704 236 branchial anlage mixed tumor glomangiopericytoma 631 melanotic neuroectodermal panniculitis-like T-cell 768, 769 glomus tumor 626–627, 631 tumor of infancy 709 lymphoma 951 breast carcinoma 73, 803–804, granular cell tumor 56–57, meningioma, ectopic 66, 69, papillary intralymphatic 805 673, 675 715–718 angioendothelioma 593– sarcomatoid 805 granular cell tumor, gingival Merkel cell carcinoma 55, 802, 594, 595 Burkitt lymphoma 949 676, 677 802 paraganglioma see calcifying fibrous granulosa cell tumor 506, 507 mesenchymal paraganglioma, pseudotumor 254 hemangioendothelioma chondrosarcoma immunohistochemistry Castleman disease 943 epithelioid 47, 597–598, 600 (extraskeletal) 57, 833– PEComa 521–522, 522 CD antigens see entries at CD epithelioid sarcoma-like 834, 834 penile myointimoma 190, 191 cell cycle markers 75–77 601, 601 mesothelioma perineurioma 668, 671, 671 cellular blue nevus 59, 61, 694 kaposiform 568 malignant 67, 70, 72, 789, pheochromocytoma 728 chordoma 66, 766, 767 PILA 593–594, 595 789, 792–794 phosphaturic mesenchymal clear cell sarcoma, hemangioma 555, 566, 570, multicystic peritoneal tumor 836 gastrointestinal 708 572, 574 (benign) 779 plasmacytoma 950 clear cell sarcoma of tendons hemangiopericytoma 333 sarcomatoid 789, 790 plexiform fibrohistiocytic and aponeuroses 56, 59, hematoma 927 metastatic carcinoma (in tumor 282 707, 708 hematopoietic cell markers 942 general/unknown prostatic carcinoma 815, 815 clear cell “sugar” tumor 520 hepatocellular carcinoma primary) 65, 812, 818– renal cell carcinoma 811, collagenous fibroma 208, 211 809 819 812–813 crystal-storing histiocytosis histiocytic markers 58, 61–62 mixed tumor/myoepithelioma reticulohistiocytoma 963–964 965, 966 histiocytic sarcoma 965 74, 759, 761 retroperitoneal fibrosis 200 dendritic reticulum cell infantile digital fibroma 256 MPNST 54, 57, 678–679, 681– rhabdoid tumor 883–885, sarcoma 965–968 infantile fibrosarcoma 294 682, 685–686 884 dermatofibrosarcoma inflammatory myofibroblastic multispecific markers 56 rhabdomyoma 50, 528–529 protuberans 46, 343–345, tumor 70, 291, 291 muscle cell markers 48–52, rhabdomyosarcoma see 345 juvenile nasopharyngeal 546–547 rhabdomyosarcoma desmoid fibromatosis, angiofibroma 265 myeloid tumor, (RMS), 242–244, 245 juvenile xanthogranuloma 63, extramedullary 954, 955 immunohistochemistry desmoplastic small round cell 962 myofibroblastoma Rosai–Dorfman disease 58, tumor 75, 880, 880 juxtaglomerular cell tumor cervicovaginal 493, 494 957, 959 diffuse large cell B-cell 633–634 palisaded 218 schwannoma 56, 73–74, 655 lymphoma 947 Kaposi’s sarcoma 46, 48, 616, myofibroma/myofibromatosis cellular 656 ectomesenchymoma 545 616 267 epithelioid 657, 658 endometrial stromal sarcoma Langerhans cell histiocytosis myopericytoma 633 gastrointestinal 661 70, 502, 503 960–961, 961 myxoid chondrosarcoma melanotic 664 endometriosis 507 leiomyoma 49–51, 73, 447, (extraskeletal) 54, 57, 831 sclerosing epithelioid endothelial/multispecific 450, 459, 460 myxoinflammatory fibrosarcoma 74, 351 markers 41–48 leiomyosarcoma 47, 49–51, 66, fibroblastic sarcoma 354 seminoma 818 ependymoma 712–713 454–455, 455 myxoma 300, 307 solitary fibrous tumor 70, epithelial markers 62–69 lipofibromatosis 258–259 cardiac 314 328–329, 329, 330 epithelioid sarcoma 47, 74, lipoma 394, 397, 401, 403–404 nerve sheath 666, 667 squamous cell carcinoma 803 755, 757, 758 liposarcoma 46, 57, 423, 429, nasal-type NK/T-cell superficial acral fibromyxoma Erdheim–Chester disease 958 431, 436 lymphoma 952 312, 312 Ewing sarcomas/PNETs see lung carcinoma nerve sheath tumors superficial angiomyxoma 307 Ewing sarcoma family of adenocarcinoma/large cell epithelioid 664 synovial sarcoma see synovial tumors, 792–793, 805, 806 MPNST 54, 57, 678–679, sarcoma, immunohistochemistry small cell 53–54, 54, 806, 807 681–682, 685–686 immunohistochemistry fasciitis lymphangioma 585 myxoma 666, 667 tenosynovial giant cell tumor intravascular 187–189 lymphangiomyoma(tosis) 59, neural/neuroendocrine 920 nodular 183–184, 188 59, 519 markers 52–57, 73–74 thyroid carcinoma 817 proliferative 193 lymphoblastic lymphoma 948 neuroblastoma see urothelial carcinoma 67–68, female genital tract stromal lymphoid markers 942 neuroblastoma, 811–813 tumors 492–493, 496– malignant fibrous immunohistochemistry immunosuppressed patients 497, 499 histiocytoma 62, 63, 71 neurofibroma 56, 647, 647, bacillary angiomatosis 578 fibromyxoid sarcoma (low- pleomorphic MFH 650–652 benign fibrous histiocytoma grade) 74, 347 (undifferentiated neuroma (palisaded 220 fibrous umbilical polyp 252–253 sarcoma) 71, 360, 362 encapsulated) 644, 644 Burkitt lymphoma 949 follicular hyperplasia 941 malignant mixed müllerian neurothekeoma 277–280, 281 EBV-associated follicular lymphoma 945 tumor 75, 505, 505 ossifying fibromyxoid tumor leiomyosarcoma 3–4, Gardner fibroma 262, 262 marginal zone lymphoma 947 57, 318 467–468, 468

1049

Index

immunosuppressed patients no oncogenic viruses 291–292 intraneural perineurioma 668,668 Kaposi’s sarcoma 611–616 (cont.) terminology 288 intravascular B-cell lymphoma appearance 613 EBV-associated lymphoma treatment 127 948–949, 949 classic form 612 947 inflammatory myxohyaline intravascular bronchioloalveolar differential diagnosis 218, 616 Kaposi’s sarcoma 612–613 tumor of distal tumor (pulmonary endemic form 612–613 malakoplakia 930 extremities epithelioid epidemic form (AIDS- Merkel cell carcinoma 798 (myxoinflammatory hemangioendothelioma) associated) 612 immunotherapy 1032 fibroblastic sarcoma) 596–597, 599 etiology (HHV8 virus) 3, 612 implants 3 352–356 intravascular fasciitis 182, 185–189, histopathology 613–616, 614– carbon fibers/graphite 911– appearance 354 190 see also penile 615 912, 912 cytology 984, 985 myointimoma nodular lesions 614–615, metal fragments 912–913, 913 differential diagnosis 354–355, histopathology 187, 190 614 polyethylene wear particles 984 intravascular pyogenic patch stage 613 910, 911 histopathology 354, 355 granuloma 557, 559 pulmonary infiltrates 612 silicone pseudolipoblasts 356 intravenous leiomyomatosis sarcomatoid 615 breast implants 409, 410, Reed–Sternberg-like cells 463–464, 464 iatrogenic form 613 953 356 intussusception, florid vascular immunohistochemistry 46, 48, joint prostheses 911, 912 inflammatory pseudotumor 288 proliferation 580, 581 616, 616 imprinting 725 inhibin 506 irritation fibroma of the oral karyotype 94–95 IMT see inflammatory INI1/INI1 (SMARCB1/ cavity 212–213, 217 Kasabach–Merritt syndrome myofibroblastic tumor SMARCB1) (SWI/SNF ischemic fasciitis 195 556–557, 567 incidence 2–3 complex) 128, 133–134, histopathology 195, 197 KBA62 (IHC marker) 61 incisional biopsy 1012–1013 545, 758, 882, 885 isocitrate dehydrogenase (IDH) keloids 201, 202 inclusion body fibromatosis see IHC marker 74, 545, 755, 884, 138, 575 keratins 62–65, 744 infantile digital fibroma 884–885 in angiosarcoma 66, 611 infantile diffuse fibromatosis insulin-like growth factor/ jaw, myxoma 315–317, 317 antibodies used in IHC 68–69 260–261, 261 receptor (IGF/IGF1R) JAZF1-SUZ12 gene fusion 123, in branchial anlage mixed infantile digital fibroma 254–256 136–137 503 tumor 768, 769 appearance 254–255, 256 intensity-modulated radiation jugulotympanic paraganglioma of complex epithelia histopathology 255–256, 256– therapy (IMRT) 1016, 737, 738 K5/K14 67–69 257 1016 juvenile aponeurotic fibroma K17 68 infantile fibrosarcoma 292–294 interdigitating reticulum cell (calcifying fibroma) 256– in epithelioid sarcoma 755 appearance 293 sarcoma 58, 702, 968 257 in GIST 66, 486 histopathology 293–294, 293– intermetatarsal compression histopathology 257, 259–260 in hemangioendothelioma 598 294 neuritis see Morton juvenile capillary hemangioma of internal squamous epithelia/ infantile hemangioma 554–555 neuroma see infantile hemangioma urothelium (K4/K13) histopathology 555, 556 intermuscular lesions 18, 20 juvenile diffuse fibromatosis 67–68 infantile myofibroma/ intra-abdominal desmoid 259–260 K3/K12 68 myofibromatosis fibromatosis 240–241, juvenile hyaline fibromatosis 253, K6/K16 68 266–268 245, 487 269–272 K15 68 cytology 984, 984 intra-articular lesions 18–20 appearance 271 of keratinizing squamous histopathology 267, 269–270 intralymphangial stromal myosis histopathology 271, 272 epithelia (K2/K9/K11) 68 infiltrative angiolipoma see endometrial stromal juvenile nasopharyngeal of keratinizing stratified (intramuscular sarcoma angiofibroma 263–265 epithelia hemangioma) 381–382 intramuscular glomus tumor 629 appearance 263–264, 263 K1 68 inflammatory fibrosarcoma 288 intramuscular hemangioma 381– histopathology 264, 264–265 K10 68 inflammatory liposarcoma 382, 562 juvenile rhabdomyoma 528, 528 in leiomyosarcoma 455 (lymphocyte-rich) appearance 564 juvenile variant DFSP (giant cell in melanoma 700–701 421, 421–422, 422 histopathology 562, 564 fibroblastoma) 342–343, in Merkel cell carcinoma 802 inflammatory malignant fibrous intramuscular leiomyosarcoma 343–344 in metastatic carcinomas 65, histiocytoma 336, 367 456 juvenile xanthogranuloma (JXG) 819 differential diagnosis 367 intramuscular lesions 18, 20 224, 961–962 in mixed tumor 759 histopathology 367, 368 intramuscular lipoma 381 appearance 962 of simple nonstratified inflammatory myofibroblastic intramuscular myxoma 299–302 histopathology 962, 963 epithelia tumor (IMT) 288–292 appearance 300, 300 immunohistochemistry 63, 962 K7 67, 611 appearance 289, 289 cytology 1000, 1001 juxtaarticular myxoma 302–303 K8/K18 65–69, 486, 598, clinical features 288–289 histopathology 300, 301–302 histopathology 303, 303–304 611, 700–701, 802 differential diagnosis 291–292, intramuscular nodular fasciitis juxtaglomerular cell tumor 633– K19 67, 598 487 183, 188 634, 634 K20 67 genetics 107, 127, 292 intraneural neurofibroma 646– JXG see juvenile in synovial sarcoma 66–67, histopathology 289–291, 290 647 see also plexiform xanthogranuloma 750, 751 cardiac variant 291 neurofibroma Ki67 (MK167) 75–76, 329 epithelioid variant 290, 291 appearance 648 kaposiform kidney tumors see also immunohistochemistry 70, histopathology 647, 648–649 hemangioendothelioma angiomyolipoma; renal 291, 291 atypia 649 567–568, 568–569 cell carcinoma, metastatic

1050

Index

glomus tumor 625 epithelioid, gynecological 463 immunohistochemistry 47, spindle cell/pleomorphic hereditary leiomyomatosis and ER-positive 72–73 49–51, 66, 454–455, 978, 979 renal cell cancer 445–446, abdominal 459 455 differential diagnosis 380–382, 461, 462–463 benign metastasizing 464– other sites 458 394, 401–402, 404, 977–979 juxtaglomerular cell tumor 465 retroperitoneal 456–457 genetics 131, 382 633–634, 634 uterine 459–463 subcutaneous/intramuscular angiolipoma 390 renal capsule PEComa 520, 520 vulvar 459 456 chondroid 404 synovial sarcoma 746 gastrointestinal 448–449 uterine 466–467, 1031 hibernoma 402 Wilms’ tumor 856, 882 intramural 449–451 venous 457 lipomatosis 387 Kimura disease 943–945, 945 muscularis mucosae leprosy 930 spindle cell/pleomorphic KIT/KIT (CD117) 451–452 Letterer–Siwe disease 960 394 immunohistochemistry 74, genetics LGFMS see fibromyxoid sarcoma hemosiderotic fibrolipomatous 484, 485, 611 angioleiomyoma 447 (low-grade) tumor 404–405 mutations gastrointestinal 450–451 LHFP (lipoma HMGA2/HMGIC hibernoma 21–22, 396–402, in GISTs 131–132, 475, uterine 462–463 fusion partner gene) 382 978–979 486–487 histopathology LHH see Langerhans cell histopathology 379–380, in mastocytosis 956 abdominal uterine-type 459, histiocytosis 380–381 and TKI sensitivity 486, 460 Li–Fraumeni syndrome 135 angiolipoma 389–390, 389 1024–1026 angioleiomyoma 446–447, limb perfusion therapy 1028 angiolipoma, cellular NGS profile 145 448–449 lip, mucosal neuroma 639, 641 389–390, 390 as a therapeutic target 132, benign metastasizing 465 lipid-rich histiocytes see chondro-osseous metaplasia 487–488 epithelioid 463 xanthogranulomatous 383 Knudson, Alfred, ‘two-hit gastrointestinal 450, 451–452 inflammation; xanthoma chondroid lipoma 402–403, hypothesis’ 108–109, 116, lipoleiomyoma 404, 460 lipid-rich rhabdomyosarcoma 402–403 137–138 pilar 446, 446 (RMS) 544 fibrolipoma 382 Krebs cycle dysfunction 138, scrotal 449 lipidized fibrous histiocytoma hemosiderotic 724–725 uterine 461–462, 461 225–226, 227 fibrolipomatous tumor immunohistochemistry 49–51, lipoblastoma 405–407 404 labia majora 73, 459, 460 appearance 406 hibernoma 399–400, angiomyofibroblastoma angioleiomyoma 447 cytology 978, 979 400–401 494–496 gastrointestinal 450 histopathology 406–407, intramuscular 381 cellular angiofibroma 496 pilar 444–446 406–407 lipoma arborescens 384 LAM see lymphangiomyoma/ scrotal 447–448 imaging 19,21 myelolipoma 390, 391 lymphangiomyomatosis visceral (ER-negative) 452 lipodystrophy (fat atrophy) 407, myxolipoma 382 laminin 72 leiomyomatosis 408 neural fibrolipoma 387 Langerhans cell histiocytosis esophageal 450 lipofibromatosis 257–260 nevus lipomatosus (LHH) 960–961 hereditary leiomyomatosis and differential diagnosis 259–260, superficialis 383 histopathology 960, 960 renal cell cancer 445–446, 283 pleomorphic lipoma immunohistochemistry 960– 461, 462–463 histopathology 258, 260–261 393–394, 397 961, 961 intravenous 463–464, 464 lipogranuloma sclerotic lipoma 398 Langerhans cells 58 peritoneal 465–466, 466 fat necrosis 407–409, 408, spindle cell lipoma 393, lapatinib 133 leiomyosarcoma (LMS) 443, 977–978, 978 394–396 large B-cell lymphoma, diffuse 452–455, 995 sclerosing 409, 409 thymolipoma 385, 386 947, 948 appearance 452–453, 453 lipoleiomyoma 404, 460 imaging 15,20–21, 23 large cell carcinoma of the lung uterine 467 lipoma 379 hibernoma 14,21–22 (metastatic) 792–793, vena cava 458 angiolipoma 388–390 immunohistochemistry 394, 805, 806 chemotherapy 1031 appearance 379, 380 397, 401, 403–404 large cell lymphoma, anaplastic in children 459 chondroid 402 lipomatoses 386–387 see anaplastic large cell cutaneous 455–456 hibernoma 399, 400 lipomatosis of the nerve (neural lymphoma (ALCL) cytology 995, 996 lipoma arborescens 383 fibrolipoma) 385–386 laryngeal inflammatory differential diagnosis 443, 457, myelolipoma 391 lumbosacral/spinal cord- myofibroblastic tumor 467, 482 neural fibrolipoma 386 associated 384–385 289 EBV-associated 3–4, 467–468, spindle cell lipoma 394 myelolipoma 390 laryngeal liposarcoma 418–419 468 thymolipoma 385 myolipoma 404 laryngeal sarcomatoid carcinoma electron microscopy 455 atypical see well-differentiated myxolipoma 977 804 epithelioid, gynecological 463 liposarcoma nevus lipomatosus superficialis Ledderhose disease see plantar gastrointestinal 457 with chondro-osseous 383 fibromatosis genetics 455 metaplasia 382–383 sclerotic 395–396 leiomyoma 443 histopathology 453–454, chondroid 402–404 spindle cell/pleomorphic 391– angioleiomyoma 446–447 453–454 clinical features 379, 384–386, 394, 978 appearance cutaneous 456 388, 390, 392, 398–399, thymolipoma 385 esophageal 450, 451 EBV-associated 468 402 visceral 384–385 retroperitoneal 459 inflammatory 455 cytology 977, 977 lipoma arborescens (synovial differential diagnosis 443, 465, pleomorphic 454 hibernoma 978–979, 980 lipomatosis) 383–384, 467 vascular form 458 myxolipoma 977, 977 383–384

1051

Index

lipoma HMGA2/HMGIC fusion WDLS myxoid type 422, lipoma 385 and crystal-storing partner gene (LHFP) 382 422–423 liposarcoma 424–425 histiocytosis 964–965, lipoma-like well-differentiated WDLS sclerosing type 420– lymphangiomyomatosis 965–966 liposarcoma 419–420, 419 421, 421 518–519, 519 diffuse large cell B-cell 947, 948 lipomatoses 386–387 imaging 22–25 meningiothelial nodules 717, extranodal marginal zone lipomatosis of the nerve dedifferentiated 6, 25 717 946–947, 947 385–387, 386–387 myxoid 17, 24 sarcomatoid carcinoma 807, follicular 945–946, 946 lipomatous hemangiopericytoma well-differentiated 23 808 genetics 946, 949, 952–954 332–333, 332 immunohistochemistry 46, 57, small cell carcinoma, Hodgkin’s disease 367, 953 lipomatous hypertrophy of the 423, 429, 431, 436 metastatic 53–54, intravascular B-cell 948–949, 949 interatrial septum 385 myxoid 24, 126–127, 416, 802–803, 806, 806–807 lymphoplasmacytic, and liposarcoma 416 430–435, 981–982 synovial sarcoma 746 amyloid tumor 936–937 appearance pleomorphic 24, 416, 435–438 lupus panniculitis 951–952 nasal-type NK/T-cell 952, 952 dedifferentiated 425, 426 round cell 24, 431, 981–982 Lyme disease 904, 905 panniculitis-like T-cell 950– myxoid 431, 431 spindle cell 423 lymph node tumors 952, 951 pleomorphic 435, 436 surgery 1014–1015, 1019–1020 angiomyomatous hamartoma plasmablastic B-cell 949–950, well-differentiated 419, 419 well-differentiated (WDLS) 444, 445 951 chemotherapy 1017 22–24, 131, 381, 416–424, endemic Kaposi’s sarcoma in precursor B-cell lymphoblastic clinical features 417–419, 979–981, 1014–1015 children 613 947–948, 948 424–425, 430–431, 435 liver tumors palisaded myofibroblastoma lymphomatoid papulosis 954 cytology angiomyolipoma 513–514, 514 213–218 lymphoplasmacytic lymphoma, myxoid/round cell 981–982, angiosarcoma 3–4, 606, 606 lymphangioendothelioma, and amyloid tumor 981 hemangioendothelioma 596– benign 585–587 936–937 well-differentiated 979–981, 597, 598 histopathology 586, 586–587 Lyon’s hypothesis 139 980 hepatocellular carcinoma 809, lymphangioendotheliomatosis lysosomal proteins 62 dedifferentiated (DDLS) 7, 24, 809 with thrombocytopenia 108, 416, 424–430 PEComa in the falciform 585–586 M-FISH (multicolor FISH) 100 differential diagnosis 381, 401– ligament 521 lymphangioma 581–585 macroscopic appearance see 402, 424, 429–430, 432–433 LMS see leiomyosarcoma acquired progressive (benign gross anatomy/ genetics 101 lobular capillary hemangioma lymphangio morphology dedifferentiated 108, 430 557–558 endothelioma) 585–587 Maffucci syndrome 573, 573, 575 myxoid 106, 126–127, histopathology 557–558, 558 appearance 584, 587 magnetic resonance imaging 434–435 intravascular pyogenic differential diagnosis 585, 587 (MRI) 13–14 pleomorphic 437–438 granuloma 557, 559 diffuse (lymphangiomatosis) angiomatous lesions 25–26, well-differentiated 102, 131, localized fibrous tumor see 587–588 27–28, 30 416–423 solitary fibrous tumor histopathology 584–585, cartilaginous tumors 34, 35, histopathology 419, 425–428, localized interdigital neuritis see 584–585 825, 826, 828, 833 431, 435–436 Morton neuroma benign collagenous fibroma 210 DDLS meningothelial-like localized tenosynovial giant cell lymphangioendothelioma elastofibroma 22 whorls 426–427, tumor 915–917, 920–921 586, 586–587 fibromatosis 34–35, 36 427–428 histopathology 916–918, 916– imaging 27, 28 lipomatous lesions 6, 15, 23–25 DDLS pleomorphic forms 917 lymphangioma circumscriptum lymphangioma 28 428 cartilaginous metaplasia 919 582 myxoma 300 DDLS resembling giant cell morphology 917 lymphangiomatosis 587, neurogenic lesions 20, 22, inflammatory MFH 426 infarction 918 587–588 30–31, 31–32 DDLS resembling various loss of heterozygosity (LOH) 143 lymphangiomyoma/ popliteal cyst 33 sarcoma types 427 low-grade fibromyxoid sarcoma lymphangiomyomatosis PVNS 21,34 DDLS see fibromyxoid sarcoma (LAM) 518–519 rhabdomyosarcoma 535, 539 rhabdomyosarcomatous (low-grade) appearance 518–519, 518 malakoplakia 930, 931 type 429 lumbosacral lipoma 384–385 histopathology 519, 519 male genital tumors see penile myxoid hibernoma-like 433 lung tumors immunohistochemistry 59, 59, fibromatosis; penile myxoid lymphangioma-like clear cell “sugar” tumor 519– 519 myointimoma; scrotal 433 520, 520 lymphedema tumors; testicular tumors myxoid postradiation differential diagnosis of angiosarcoma in malignant eccrine spiradenoma therapy 435 mesothelioma 792–793 lymphedematous 763 myxoid round cell 434 glomus tumor 625 extremities 4, 603–604, 604 malignant fibrous histiocytoma myxoid typical forms 432 hemangioendothelioma 596– in the obese patient 409–411, (MFH) pleomorphic 437 597, 599 411 clinical features 357, 362, 367, pleomorphic epithelioid inflammatory myofibroblastic lymphoblastic lymphoma 369 form 438 tumor 288–289 947–948, 948 cytology spindle cell 423 large cell carcinoma, metastatic lymphoma 941 giant cell MFH 989 WDLS inflammatory type 792–793, 805, 806 anaplastic large cell pleomorphic MFH 988, 988 421–422, 421–422 leiomyoma 452 cutaneous 954 differential diagnosis 348, WDLS lipoma-like 419–420, benign metastasizing 464– noncutaneous 952–954, 953 358–359, 364, 366–367, 419 465, 465 Burkitt 949, 950 988

1052

Index

electron microscopy 357–358, geographic necrosis 681 differential diagnosis 9, 697, Merkel cell polyomavirus (MCV) 362 glandular differentiation 684 701–702 801–802 genetics 134, 359, 362–364 in a neurofibroma 679 genetics 702 merlin (NF2 protein) 133 giant cell 368–369, 989 with pheochromocytoma histopathology 696–698 659–660 histopathology 686, 732 osteoclastic giant cells 699 mesenchymal chondrosarcoma, giant cell MFH 369, 370 rhabdomyosarcomatous pleomorphism 699 extraskeletal (MCHSA) inflammatory MFH 367, 368 differentiation (Triton resembling hematopoietic 832–834 myxoid MFH 357, 359–361 tumor) 683 tumors 700 appearance 833 pleomorphic MFH 362, vascular proliferation 681 various patterns 697–698 cytology 1000, 1000 363–364 imaging 11–12, 29, 31, 32 immunohistochemistry 58–61, histopathology 832–833, 833 imaging 16–20, 21 immunohistochemistry 54, 57, 60, 74, 699–701, 700–701 imaging 832, 833 immunohistochemistry 62, 63 678–679, 681–682, S100 56, 699, 701 immunohistochemistry 57, pleomorphic MFH 71, 360, 685–686 melanoma of soft parts see clear 833–834, 834 362 surgery 1018 cell sarcoma of tendons mesenchymoma 1 inflammatory 336, 367 transformed neurofibroma 652 and aponeuroses mesna 1029 myxoid 336, 356–359, 985–986, Triton tumor 683 melanotic neuroectodermal mesodermal stromal polyp 1018 malignant rhabdoid tumor see tumor of infancy 491–493, 492 pleomorphic 336, 359–364, rhabdoid tumor, (melanotic progonoma) mesothelial proliferations 987–988 extrarenal 708–709, 991 (benign or precancerous) malignant glomus tumor 626, malignant tenosynovial giant cell cytology 991 adenomatoid tumor 777, 778 630 tumor (TSGT) 921–922, histopathology 709, 710 differential diagnosis 777 malignant granular tumor 922 melanotic schwannoma 661–664 789–792, 791 675–676, 676 MALT lymphoma (extranodal appearance 662, 662 multicystic peritoneal malignant histiocytic tumors marginal zone differential diagnosis 664, 702 mesothelioma 778–780 (histiocytic sarcoma) 965, lymphoma) 946–947, 947 histopathology 662–663, 663– appearance 779 966 mammary-type 664 cellular nodules 789, 791 malignant histiocytosis see myofibroblastoma see melphalan 1027–1028 histopathology 779–780, 779 anaplastic large cell breast myofibroblastoma MEN2a/MEN2b syndromes well-differentiated papillary lymphoma marginal zone lymphoma 946– (multiple endocrine mesothelioma 780–781, malignant melanoma see 947, 947 neoplasia) 781, 789–790, 791 melanoma, metastatic MART-1 (melan A) 58, 60 mucosal neuroma/ mesothelioma, malignant malignant mesothelioma see Masson tumor (papillary ganglioneuroma 639, 641 782–794 mesothelioma, malignant endothelial hyperplasia) paraganglioma 725–726 appearance 783 malignant mixed müllerian 577–578, 577–578 meningeal hemangiopericytoma peritoneal 784 tumor 503–505 mastocytoma 956, 956 331 pleural 784 appearance 504, 504 mastocytosis 956, 957 meningeal solitary fibrous tumor differential diagnosis 789 histopathology 504, 504–505 Mazabraud syndrome 300 324–325, 331 vs benign/reactive immunohistochemistry 75, McCune–Albright syndrome 300 meningioma, ectopic 713 mesothelial proliferation 505, 505 MCHSA see extraskeletal cytology 990–991, 991 777, 789–792, 791 malignant mixed tumor mesenchymal differential diagnosis 718 vs carcinoma 789, 792–793, myoepithelial carcinoma 50, 702 chondrosarcoma histopathology 792–793 myoepithelioma 761–762 MDM2/MDM2 (murine double paraspinal 716, 716 vs mesenchymal tumors 793 malignant perineurioma 671–672 minute) pulmonary 717 vs vascular tumors 793–794 malignant peripheral nerve amplification 101, 131 scalp 714–715, 715 electron microscopy 789, 790 sheath tumor (MPNST) liposarcoma 416–424, 430 sinonasal 716, 716 epidemiology 782–783 (malignant schwannoma) and p53 135–136 immunohistochemistry 66, 69, etiology 782–783 676–682 medallion-like dermal dendrocytic 715–718 genetics 794 with angiosarcoma 607, 683 hamartoma 345 metastatic 717 histopathology appearance 678, 679, 684 media for cell culture 93 paraspinal 716 biphasic 786–787, 787 cytology 992, 992–993 for transport 93 pulmonary 717 deciduoid 785–786, 786 differential diagnosis 680–684, mediastinal fibrosis 200 scalp 714–715 desmoplastic 788–790, 788 702, 993 mediastinal liposarcoma, well- sinonasal, ear and orbital epithelioid 785, 786 epithelioid differentiation 684– differentiated 418 716–717 sarcomatoid 787–788 686 mediastinal paraganglioma 734 meningiothelial hamartoma 787–788 with ganglioneuroma or mediastinal thymolipoma 714–715, 715 tubulopapillary 784–785, pheochromocytoma 686 385–386, 385 meniscal cyst 32–33 784 genetics 108–110, 117, 682 Meissner-like bodies 56, 651 Merkel cell carcinoma 798–803 tubulopapillary glandular epithelial melan A (MART-1) 58, 60 appearance 799, 799 adenomatoid-like 785 differentiation 54, 683– melanocytes 56 clinical features 798–799 immunohistochemistry 67, 70, 684 melanoma, desmoplastic differential diagnosis 802–803 72, 789, 789, 792–794 granular cell tumor 675–676, 702–704 etiology 798, 801–802 sarcomatoid 789, 790 675–676 histopathology 702–703, 703 histopathology 799–801, 800– peritoneal 783, 784, 792–793 histopathology 678, 680 immunohistochemistry 704, 801 pleural 783, 784 epithelioid differentiation 704 immunohistochemistry 55, MET (hepatocyte growth factor 685, 685 melanoma, metastatic 695–702 802, 802 receptor) 130, 136–137

1053

Index

metabolism of cancer cells 138 mitosis/karyorrhexis index (multilocular peritoneal myofibroblastic sarcoma metal foreign bodies 3, 607–608 (MKI) 873 inclusion cyst) 778–780 (myofibrosarcoma) 352, synovial reaction to prosthetic mixed tumor see also branchial appearance 779 353 fragments 912–913, 913 anlage mixed tumor; cellular nodules 789, 791 myofibroblastoma metal-containing fixatives 8 malignant mixed histopathology 779–780, 779 breast 218–220 metaplasia müllerian tumor multinodular (myxoid) cytology 983, 983–984 bone 847, 848 myoepithelial carcinoma 50, myofibroblastoma see histopathology 218–219, 220 lipoma with metaplastic 702 neurothekeoma cervicovaginal 493, 493 cartilage and bone myoepithelioma 758–761 multiple endocrine neoplasia palisaded 213–218, 218 382–383, 383 cytology 1000, 1001 types 2a and 2b (MEN2a/ histopathology 216–218, 219 metastatic disease histopathology 759, 760 MEN2b) myofibroblasts 49–50, 181–182 adrenocortical carcinoma 60, immunohistochemistry 58, mucosal neuroma/ myofibroma/myofibromatosis 815, 816 74, 759, 761 ganglioneuroma 639, 641 adult 267, 270–271 angiomatoid fibrous malignant 761–762 paraganglioma 725–726 infantile 266–268 histiocytoma 283 MLS see myxoid liposarcoma multiple hamartoma syndrome cytology 984, 984 benign fibrous histiocytoma 221 molecular genetics see genetics 211 histopathology 267, 269–270 benign metastasizing monomorphous round cell RMS multiple myeloma (disseminated myofibrosarcoma (myofibroblastic leiomyoma 464–465, 465 see alveolar plasmacytoma) 949 sarcoma) 352, 353 breast carcinoma 73, 803–805, rhabdomyosarcoma multiplex PCR 141, 143 myogenesis 531, 534 805 monophasic (spindle cell) muscle myogenic regulatory factor endometrial stromal sarcoma synovial sarcoma 52, IHC markers 48–52 (MyoD1) 51–52, 546, 899 501 748–749, 749, 751 myogenesis 531, 534 myogenic sarcoma 364 gastrointestinal carcinoma Morton neuroma 638 muscle-specific actin (HHF-35) myogenin 51–52, 546, 547 808–809 histopathology 639 49, 546 myoglobin 52, 546 GISTs 476, 481, 483, 1024– imaging 30, 31 muscularis mucosae leiomyoma myolipoma 404 1027 mouth tumors 451–452, 452 myopericytoma 632–633, 633–634 hepatocellular carcinoma 809, giant cell fibroma 213, 218 musculoaponeurotic myosin 50 809 gingival fibromatosis 253, 253– fibromatosis see desmoid myositis IHC profiles of carcinomas 65, 254 fibromatosis focal 194, 531, 531 812, 818–819 irritation fibroma 212–213, 217 MYC/MYCN/MYC/MYCN proliferative 194–195, 195 liposarcoma 424–425, 431 mucosal neuroma 639, 641 gene amplification 130 myositis ossificans 841–845 lung carcinoma pulse granuloma 929, 929 angiosarcoma 611 cytology 999, 999–1000 large cell 792–793, 805, 806 rhabdomyoma 529, 529–530 FISH 98–99, 99, 877 histopathology 842–843, 844 small cell 53–54, 802–803, solitary fibrous tumor 324 neuroblastoma 99, 130, 875– imaging 12, 842, 843 806, 806–807 MPNST see malignant peripheral 876, 877 myxofibrosarcoma (myxoid melanoma see melanoma, nerve sheath tumor rhabdomyosarcoma 131 MFH) 336, 356–359 metastatic MRI see magnetic resonance gene translocation in Burkitt appearance 358 meningioma 717 imaging lymphoma 949 classification 356–357 ovarian carcinoma 815–816 mTOR signaling pathway 128, MYC/MYCN expression in clinical features 357 prostatic carcinoma 813–815, 136 neuroblastoma cytology 985–986, 986 814 inhibitors 133, 518 as an IHC marker 873, 875, differential diagnosis 348, renal cell carcinoma see renal MUC4 protein 74 876 358–359 cell carcinoma, metastatic mucin, production by myxomas and prognosis 878 genetics 359 seminoma 817–818, 818 299 mycobacterial pseudotumor histopathology 357 squamous cell carcinoma 758, mucinosis, focal cutaneous 929–930, 930 high-grade 361 803, 803–804 mucinosis 310, 311 mycobacterial synovitis 907–908, intermediate-grade 360–361 thyroid carcinoma 816–817, 817 mucocele 308, 310 909 low-grade 359–360 treatment 1029–1030 mucosa-associated lymphoid myeloid tumor, extramedullary imaging 16–20 unknown primary 818–819 tissue (MALT) (myeloid sarcoma) 954 treatment 1018 urothelial carcinoma 67–68, lymphoma (extranodal histopathology 954–955, 954 myxoid chondrosarcoma 811–813 marginal zone immunohistochemistry 954, 955 (extraskeletal, EMC) MFH see malignant fibrous lymphoma) 946–947, 947 sclerosing 424 830–832 histiocytoma mucosal ganglioneuroma 639, 641 myelolipoma 390, 391 appearance 830 Mibelli angiokeratoma 560 mucosal neuroma 639, 641 histopathology 390, 391 cytology 1006, 1007 Michaelis–Gutman bodies 931 müllerian adenosarcoma 505 myeloperoxidase 954 genetics 126, 832 microphthalmia transcription müllerian mixed tumor see myocutaneous flaps 1013–1014 histopathology 830, 831–832 factor (MITF) 58, 60 malignant mixed MyoD1 (myogenic regulatory hybrid with synovial sarcoma microRNA (miRNA) 116–117, müllerian tumor factor) 51–52, 546, 899 750 129, 136 multicentric Castleman disease myoepithelial carcinoma 50, 702 imaging 830, 830 microsatellite markers 143 943 myoepithelioma 758–761 immunohistochemistry 54, 57, microvenular hemangioma 575, multicentric reticulohistiocytosis cytology 1000, 1001 831 576 964, 964 histopathology 759, 760 myxoid cyst, cutaneous 305–306, midostaurin 128 multicolor FISH (M-FISH) 100 immunohistochemistry 74, 306, 307 Milroy disease 603 multicystic peritoneal 759, 761 myxoid lipoma (myxolipoma) mitochondrial conditions 386, 387 mesothelioma malignant 761–762 380, 382, 977, 977

1054

Index

myxoid liposarcoma (MLS) 416, nail bed tumors (glomus tumor) neural and neuroendocrine IHC nasal glioma 710 430–435 624 markers 52–57 neuroendocrine tumors 806 see appearance 431, 431 nasal glioma 710 neurilemmoma see schwannoma also Merkel cell clinical features 430–431 nasal glomangiopericytoma 631– neuroblastoma 867–879 carcinoma; cytology 981–982, 981 632, 632 appearance 871, 872 pheochromocytoma; differential diagnosis 301, 432– nasal-type NK/T-cell lymphoma ganglioneuroma 872 small cell carcinoma of 433, 982 952, 952 catecholamines 870 the lung (metastatic) genetics 106, 126–127, 434– nasopharyngeal angiofibroma, classification 867–870, 873, 874 neurofibroma 644–652 435 juvenile 263–265 clinical features 870–871 appearance histopathology 431, 432 appearance 263–264, 263 differential diagnosis 874, 878– cutaneous 653 hibernoma-like 433 histopathology 264, 264–265 879 intraneural 648 lymphangioma-like 433 NB84 (neuroblastoma marker) electron microscopy 874, 875 plexiform 649 postradiation therapy 435 55,55–56 genetics 130–131, 875–877 cellular 645–646, 652 round cell form 434 NCAM (CD56) 56, 806 MYCN amplification 99, cutaneous 644–645 imaging 17, 24, 24 NCOA2 gene fusion 125, 834 130, 875–876, 877 cytology 990, 990 immunohistochemistry 431 neck paraganglioma 734–737 and prognosis 876–878 differential diagnosis 345, 652 myxoid malignant fibrous appearance 735 histopathology 871–873 diffuse 648, 652 histiocytoma see histopathology 735–737, 736 in bone marrow 878 genetics 108–110 myxofibrosarcoma immunohistochemistry 54, composite 870, 870 histopathology myxoid smooth muscle uterine 737 congenital 871 cellular 646 tumors 463 necrobiotic granuloma differentiating 868, 869, 874 cutaneous 645, 645–646 myxoid well-differentiated pseudorheumatoid nodule ganglioneuroblastoma 868– diffuse 648, 651 liposarcoma 422, 422– (deep granuloma 869, 869–870, 872, 874 intraneural 647, 648–649 423 annulare) 927–929, 928 ganglioneuroma 868, 869, pigmented 651 myxoinflammatory fibroblastic rheumatoid nodule 905 872, 872 plexiform 650 sarcoma see acral necrotizing fasciitis 197–199 ganglioneuroma, mucosal hybrid tumors 660 myxoinflammatory appearance 198, 198 639, 641 imaging 30–31 fibroblastic sarcoma histopathology 199, 199 lymphocytic infiltration in immunohistochemistry 56, myxolipoma (myxoid lipoma) necrotizing granuloma 758 opsoclonus-myoclonus 647, 647, 650–652 380, 382, 977, 977 in renal cell carcinoma 811 875 intraneural 646–647 myxoma 299 needle biopsy see core biopsy; neuropil/Homer Wright malignant transformation 652 cardiac 313–315 fine-needle aspiration rosettes 868, 872, 874 pigmented 648–650 glandular epithelial nephrogenic systemic fibrosis nucleoli 873 plexiform 31, 647 differentiation 314, 316 (gadolinium-associated) poorly differentiated 868, neurofibromatosis type 1 (NF1) histopathology 314, 314–316 936, 936 868, 873–874 19, 653 Carney complex 306, 308–310 nerve growth factor receptor p75 salt and pepper nuclei 872 café au lait spots 653 cutaneous 306–310 56 staging 878 genetics 108–110, 132–133, histopathology 307, 308–309 nerve resection 1013 undifferentiated 867, 868 637, 653 differential diagnosis 300–301, nerve sheath cellular anatomy history 867 GIST 483–484, 484, 653 307–308, 312–313, 317, 637–638 imaging 871 glomus tumor 631 666 nerve sheath myxoma 665–666 immunohistochemistry 53, imaging 22 intramuscular 299–302 histopathology 666, 666–667 875, 876 MPNST 652, 676 appearance 300, 300 immunohistochemistry 666, MYC/MYCN 873, 875, 876 pheochromocytoma 725–726 cytology 1000, 1001 667 NB84 55,55–56 neurofibromatosis type 2 (NF2) histopathology 300, 301–302 not neurothekeoma 277, 280– neurofilaments 55 133, 637, 659–660, 669 juxtaarticular 302–303, 303– 281, 281, 665–666 synaptophysin 53, 54, 876 neurofibrosarcoma see malignant 304 nerve sheath tumor-associated tyrosine hydroxylase 875, peripheral nerve sheath Mazabraud/McCune–Albright angiosarcoma 607, 659, 876 tumor (MPNST) syndromes 300 683 prognosis 871, 876–878 neurofilament (NF) proteins nerve sheath 665–666 nerve sheath tumors see also staging 878 54–55 histopathology 666, 666–667 granular cell tumor; neuroblastoma-in-situ 867 neuroma immunohistochemistry 666, malignant peripheral neuroblastoma-like schwannoma Morton 30, 31, 638, 639 667 nerve sheath tumor 657–658, 659 mucosal 639, 641 not neurothekeoma 277, (MPNST); nerve sheath neuroectodermal tumors see also pacinian 640–641, 642 280–281, 281, 665–666 myxoma; neurofibroma; cellular blue nevus; palisaded encapsulated 642– odontogenic 315–317, 317 neuroma; perineurioma; ependymoma; 644, 643–644 myxomatous tumors, cystic schwannoma gastrointestinal clear cell traumatic 30, 639, 640 appearance 34 differential diagnosis 345, 652, sarcoma; melanoma; neuromuscular hamartoma myxopapillary ependymoma 711, 656, 661, 664 meningioma (choristoma) 530, 530, 740 imaging 11–12, 29, 31 gastrointestinal clear cell 638, 638 cytology 991–992, 992 epithelioid 664, 665 sarcoma 708, 709 neuropil 868, 872, 874 histopathology 711, 712 hybrid 660 gliomatosis 710–711, 711 neurosecretory granules in PNET nested PCR 123, 141 melanotic neuroectodermal cells 861, 861 N-cadherin 70 neural fibrolipoma 385–386, tumor of infancy 708– neurothekeoma 277–282 NAB2-STAT6 gene fusion 330 386–387 709, 710, 991, 991 clinical features 277–278

1055

Index

neurothekeoma (cont.) nutlins 131 osteomalacia, oncogenic 834–837 parachordoma 759–761 differential diagnosis 224, 280– osteosarcoma, extraskeletal 847– paraffinoma 409 282 obese patients, massive localized 849 paraganglia 723, 723 not nerve sheath myxoma lymphedema 409–411, histopathology 848–849, 849 paraganglioma 723–740 see also 277, 280–281, 281, 665 411 imaging 848, 848 ganglioneuroma; histopathology 278, 279–281 ochronosis 913, 914 immunohistochemistry 74–75, neuroblastoma fascicular component 280 odontogenic myxoma 315–317, 849 appearance 729, 735 mixed multinodular 279 317 ovarian tumors see also adult Carney triad 482 immunohistochemistry 277– OFT see ossifying fibromyxoid granulosa cell tumor cauda equina 738–740 280, 281 tumor carcinoma 815–816 classification 723 myxoid see nerve sheath omental GISTs 482 dysgerminoma 818 differential diagnosis 737, 740, myxoma oncogenes 115, 130–131 see also germ cell tumors with 900 nevoid basal cell carcinoma ALK/ALK; MYC/MYCN/ angiosarcoma 607 gangliocytic (duodenal) 737– syndrome (Gorlin MYC/MYCN 738 syndrome) 529 IDH1/IDH2 138, 575 p15(CDKN2b) 135 genetics 724–726 nevus flammeus (port wine stain) KIT 131–132, 145, 486–487 p16 (CDKN2a) 77, 134–135, 682 histopathology 575–576 MDM2 101, 131, 135–136, p21 (CDKN1a) 135 carotid body 735–737, 736 nevus lipomatosus superficialis 416–424, 430 p27 76 cauda equina 739, 739 383, 383 RAS family 115–116 p53 (TP53) combined next-generation sequencing oncogenic osteomalacia 834–837 atypical fibroxanthoma 366 pheochromocytoma and (NGS) 123, 144–145 oncogenic viruses see viruses, cell cycle regulation 135–136 ganglioneuroma 732 NF1 see neurofibromatosis type 1 oncogenic immunohistochemistry 76 gangliocytic duodenal 738, NF2 see neurofibromatosis type 2 oncomir see microRNA rhabdomyosarcoma 548 739 NFATC2-EWSR1 gene fusion 865 Ondine’s curse 875 as a therapeutic target 131 jugulotympanic 738 NGS (next-generation oral tumors p75 (nerve growth factor pheochromocytoma 726, sequencing) 123, 144–145 giant cell fibroma 213, 218 receptor) 56 727 NK/T-cell lymphoma 952, 952 gingival fibromatosis 253, 253– pacinian neuroma 640–641, 642 pheochromocytoma-like NKX2.2 (IHC marker) 862 254 paclitaxel 1018 retroperitoneal 730 nodular fasciitis 182–185 irritation fibroma 212–213, palisaded encapsulated neuroma retroperitoneal 728–729, appearance 183, 185 217 642–644 729–731 clinical features 182–183 mucosal neuroma 639, 641 histopathology 642–643, 643 urinary bladder 733, 733 cranial fasciitis 182, 249–251 pulse granuloma 929, 929 immunohistochemistry 644, 644 immunohistochemistry cytology 982, 982 rhabdomyoma 529, 529–530 palisaded myofibroblastoma cauda equina 739–740 differential diagnosis 184–185, solitary fibrous tumor 324 213–218 gangliocytic duodenal 738 224, 251, 982 orbital tumors appearance 218 neck 54, 737 genetics 185 giant cell angiofibroma 330 histopathology 216–218, 219 pheochromocytoma 728 histopathology 183, 185–186 hemangioma 559 palisaded-vacuolated type GIST retroperitoneal 55, 732 cranial fasciitis 250, 250–251 meningioma 716 478, 479 urinary bladder 734, 734 early/late forms 186 solitary fibrous tumor 224, 324 palmar fibromatosis jugulotympanic 737 intramuscular 183, 188 organizing hematoma 926–927 clinical features 233 malignant 726–728 pseudosarcomatous appearance 926, 926 differential diagnosis 237 neck/carotid body 734–737 187 histopathology 926–927, 927 genetics 237 pheochromocytoma 723, repair variant 183, 185, 187 imaging 33 histopathology 234–235, 235 726–728 imaging (cranial fasciitis) 250 Ormond’s disease immunohistochemistry 236 with MPNST 686, 686, 732 immunohistochemistry (retroperitoneal fibrosis) treatment 234 retroperitoneal (extra-adrenal) 183–184, 188 199–201 pancreatic adenocarcinoma 809 723–724, 728–729 terminology 182 histopathology 200, 200–201 panniculitis-like T-cell syndromic forms 724–726 nodular mesothelial hyperplasia Oroya fever (verruga peruana) lymphoma 950–952, 951 thoracic 734 789, 791 579 panniculitis ossificans (fasciitis urinary bladder 729–734 nodular tenosynovitis see ossifying fasciitis (panniculitis ossificans) 182–183, paraneoplastic syndromes tenosynovial giant cell ossificans) 182–183 841 in DSCRT 882 tumor ossifying fibromyxoid tumor papillary endothelial hyperplasia in neuroblastoma 870–871 noninvoluting congenital (OFT) 317–320 (Masson tumor) 577–578, oncogenic osteomalacia hemangioma 556–557 appearance/X-ray image 319 577–578 834–837 NPM-ALK gene fusion 127 genetics 318 papillary hemangioma 565 paraspinal tumors NR4A3-EWSR1 gene fusion 126, histopathology 317–318, 319– papillary intralymphatic meningioma 716, 716 832 320 angioendothelioma paraganglioma 734 NTRK3-ETV6 gene fusion 124, immunohistochemistry 57, (PILA, Dabska tumor) paratesticular serous papillary 127–128, 294 318 593–594 carcinoma 793, 793 nuchal fibrocartilaginous osteoarthritis 905–906, 907 appearance 593 paratesticular spindle cell pseudotumor 207–208, osteoclastic giant cells histopathology 593, 594 rhabdomyosarcoma 208 in melanoma 699 immunohistochemistry 595 535–539 nuchal-type fibroma 206–207, in MFH 370, 989 papillary mesothelioma, well- parosteal lesions 182, 845–847 207 in PMT 836 differentiated 780–781, PAS (periodic-acid–Schiff) nuclear medicine 11–12, 29 osteolipoma 382–383, 383 781, 789–790, 791 positive cells

1056

Index

in alveolar soft part sarcoma malignant 671–672 pigmented villonodular synovitis pleomorphic rhabdomyosarcoma 897, 898 perineural cells 637 (PVNS) see tenosynovial (RMS) 541–542 in Ewing sarcoma 859–860 retiform (reticular) 670 giant cell tumor cytology 994, 995 PATZ1-EWSR1 gene fusion 865 sclerosing 668–670 pilar leiomyoma histopathology 542, 543 PAX-FOXO1A (FKHR) gene soft tissue (other) 670–671 (piloleiomyoma) 444– immunohistochemistry 543 fusion 129, 535, 539–540, periodic-acid–Schiff (PAS) 446, 446 pleomorphic undifferentiated 542, 547 positive cells PLAG1 gene fusion 125, 407 sarcoma see pleomorphic pazopanib 1030 in alveolar soft part sarcoma planar xanthoma (xanthelasma) malignant fibrous PBX1-EWSR1 gene fusion 126, 897, 898 932 histiocytoma 760–761 in Ewing sarcoma 859–860 plantar fibromatosis pleural mesothelioma 783, 784 PCR see polymerase chain periosteal fasciitis 182, 845–847 clinical features 233–234 see also mesothelioma, reaction peripheral nerve sheath tumors differential diagnosis 237 malignant PDGFB-COL1A1 gene fusion (PNST) see also granular genetics 237 pleural plaque 791 128–129, 345 cell tumor; malignant histopathology 235–236, 236– pleural solitary fibrous tumor PDGFRA (platelet-derived peripheral nerve sheath 237 324 growth factor alpha tumor (MPNST); nerve immunohistochemistry 236 plexiform fibrohistiocytic tumor receptor) 132, 475, 486, sheath myxoma; treatment 234 282–283 1024 neurofibroma; neuroma; plaque-like CD34-positive differential diagnosis 224, 281– targeted therapy see imatinib perineurioma; dermal fibroma 345 283, 368 mesylate schwannoma plasmacytoma 949–950, 951 histopathology 282–283, 282 PDGFRB (platelet-derived differential diagnosis 345, 652, plastic induration of the penis plexiform neurofibroma 31, 647 growth factor beta 656, 661, 664 (penile fibromatosis) appearance 649 receptor) 242 imaging 11–12, 29, 31 237–238, 238 histopathology 650 PECAM-1 (CD31) 41–44, 610 peritoneal leiomyomatosis platelet-derived growth factor plexiform schwannoma 656–657, PEComa (perivascular 465–466, 466 alpha receptor 658, 661 epithelioid cell tumor) peritoneal mesothelioma 783 (PDGFRA) 132, 475, 486, plywood fibroma (sclerotic 513 see also appearance 784 1024 fibroma) 211, 214 angiomyolipoma; differential diagnosis 792–793 targeted therapy see imatinib PMT see phosphaturic lymphangiomyoma/ perivascular epithelioid cell mesylate mesenchymal tumor lymphangiomyomatosis tumor see PEComa platelet-derived growth factor (PMT) of mixed abdominal sites 521–522 pesticides 3 beta (PDGFB) connective tissue variant appearance 520 Peyronie’s disease (penile gene fusion with COL1A1 PNET (primitive clear cell “sugar” tumor 519– fibromatosis) 237–238, 128–129, 345 neuroectodermal tumor) 520 238 PDGFB receptor in desmoids see Ewing sarcoma family cutaneous 522 phagolysosomes 673 242 of tumors differential diagnosis 465, 517, pheochromocytoma 723, pleomorphic fibroma of skin PNL2 (IHC marker) 61 523, 900 726–728 211–212, 215 PNST (peripheral nerve sheath genetics 513, 517–518, 523 histopathology 726, 727 pleomorphic hyalinizing tumors) see also granular histopathology 521, 521–522 immunohistochemistry 728 angiectatic tumor 369– cell tumor; malignant clear cell “sugar” tumor 519, malignant 726–728 372, 371 peripheral nerve sheath 520 with MPNST 686, 686, 732 pleomorphic leiomyosarcoma tumor (MPNST); nerve immunohistochemistry 521– syndromic 725–726 453–454, 454 sheath myxoma; 522, 522 pheochromocytoma of adrenal pleomorphic lipoma 391–394 neurofibroma; neuroma; renal capsule 520 scaled score (PASS) appearance 394 perineurioma; uterine 520–521 726–727 cytology 978, 979 schwannoma pediatric tumors see childhood PHF1 gene fusion 125, 318 histopathology 393–394, 397 differential diagnosis 345, 652, tumors phleboliths 25, 574 immunohistochemistry 394, 656, 661, 664 pelvic desmoids 240 phosphaturic mesenchymal 397 imaging 11–12, 29, 31 pelvic lipomatosis 386–387 tumor (PMT) of mixed pleomorphic liposarcoma 24, podoplanin (D2-40) 47 penile fibromatosis 237–238, 238 connective tissue variant 416, 435–438 POEMS syndrome 564–565 penile myointimoma 189–190, 191 834–837 appearance 435, 436 polyethylene wear particles 910, periarticular lesions 18, 20 see appearance 835 histopathology 435–436, 437 911 also synovial sarcoma histopathology 835–837, epithelioid form 438 polymerase chain reaction (PCR) perineurioma 666–672 835–836 pleomorphic malignant fibrous 141–142 differential diagnosis 668–672 imaging 834, 835 histiocytoma (MFH) allele-specific PCR 141 genetics 669, 671 immunohistochemistry 836 (pleomorphic amplification of microsatellite histopathology photography of tumors 7 undifferentiated sarcoma) markers 143 intraneural 668, 668 PHOX2B (IHC marker) 875 336, 359–364, 987–988 multiplex PCR 141, 143 retiform 670 PI3K/AKT/mTOR signaling clinical features 362 nested PCR 123, 141 sclerosing 668, 669 pathway 128, 133, 136, cytology 988, 988 RACE 119–123, 141 soft tissue (other) 670, 670 518 differential diagnosis 364, 988 real-time PCR 141–143 hybrid tumors 660 pigmented DFSP (Bednar tumor) genetics 134, 362–364 RT-PCR 119–123, 141, 977 immunohistochemistry 668, 342, 342 histopathology 362, 363–364 polyvinylpyrrolidone (PVP) 671, 671 pigmented neurofibroma immunohistochemistry 71, accumulation 933–934, intraneural 668 648–650, 651 360, 362 933–934

1057

Index

poorly differentiated psammomatous melanotic tumoral calcinosis 838 reticulohistiocytosis, multicentric neuroblastoma 868, 868, schwannoma see radionuclide imaging 11–12, 29 964, 964 873–874 melanotic schwannoma radiotherapy reticulum cells 968 poorly differentiated synovial pseudogout 910 adjuvant/neoadjuvant 1015– dendritic reticulum cell sarcoma 750, 750 histopathology 903, 910, 910 1016 sarcoma 965–968, 967 popliteal (Baker) cyst 17,31–32, tophaceous (focal) form 839, chemoradiotherapy 1028– fibroblastic reticulum cell 33 841 1029 sarcoma 968 port wine stain 575–576 tophaceous (focal) 838–839, desmoid tumors 1031 interdigitating reticulum cell positron emission tomography 840 retroperitoneal sarcoma sarcoma 58, 702, 968 (PET) 12 pseudo-Kaposi’s sarcoma 1020 retiform hemangioendothelioma postradiation angiosarcoma/ (acroangiodermatitis) adverse effects 3, 1015–1016 594–595, 595 atypical vascular lesions 580, 580 rapidly involuting congenital retiform (reticular) perineurioma 603, 604 pseudolipoblasts 356, 359 hemangioma 556–557 670, 670 post-traumatic cyst of soft tissue pseudomyogenic RAS family oncogenes 115–116 retinal anlage tumor (melanotic see organizing hematoma hemangioendothelioma RB1/RB1 (retinoblastoma) 76, neuroectodermal tumor POU5F1-EWSR1 gene fusion 599–601, 601 116, 134 of infancy) 708–709, 991 126, 760–761, 865 pseudorheumatoid nodule (deep reactive mesothelial proliferation cytology 991 PR (progesterone receptor) 73, granuloma annulare) 789–792, 791 histopathology 709, 710 1031 927–929, 928 reactive vascular proliferations retinoblastoma gene/product precursor B-cell lymphoblastic pseudosarcomatous fasciitis see 578 (RB1/RB1) 76, 116, 134 lymphoma 947–948, 948 nodular fasciitis acroangiodermatitis 580, 580 retroperitoneal fibrosis 199–201 primary neuroendocrine pseudosarcomatous fibrous angioendotheliomatosis 579– histopathology 200, 200–201 carcinoma of the skin see histiocytoma 226–228, 580 retroperitoneal leiomyoma, ER- Merkel cell carcinoma 227 bacillary angiomatosis 578– positive 73, 459, 459 primitive neuroectodermal pseudosarcomatous stromal 579, 579 retroperitoneal leiomyosarcoma tumor (PNET) see Ewing polyp 492, 492 florid proliferation related to 456–457 sarcoma family of tumors PTCH1 gene 529 intestinal intussusception retroperitoneal liposarcoma, PRKAR1A (protein kinase A) pulmonary tumors see lung 580, 581 well-differentiated 417– gene 309–310, 315 tumors superficial vascular 418, 424 progesterone receptor (PR) 73, pulse granuloma 929, 929 pseudoaneurysm 580, 581 retroperitoneal paraganglioma 1031 PVP (polyvinylpyrrolidone) verruga peruana 579 (extra-adrenal) 723–724, progressive angioma (acquired accumulation 933–934, real-time PCR 141–143 728–729 tufted angioma) 566–567, 933–934 receptor tyrosine kinases see histopathology 728–729 568 pyogenic granuloma (lobular tyrosine kinase receptors combined proliferative fasciitis 190–194 capillary hemangioma) reconstructive surgery 1013– pheochromocytoma and appearance 192–200, 193 557–558 1014 ganglioneuroma 732 cytology 982 histopathology 557–558, 558 rectal GISTs 481, 482 nuclear atypia 731 histopathology 192–193, 193– intravascular 557, 559 recurrent digital fibroma see organoid patterns 729–731 194 pyrosequencing 144 infantile digital fibroma pheochromocytoma-like proliferative myositis 194–195, Reed–Sternberg-like cells 356 730 195 quantitative-PCR (Q-PCR, real- regorafenib 1025–1026 pigmented 731 prostatic carcinoma 125, 813– time PCR) 141–143 Reichel disease see synovial immunohistochemistry 55, 815 chondromatosis 729, 732 histopathology 814, 814 RACE (rapid amplification of renal cell carcinoma, metastatic retroperitoneal sarcoma 1019– immunohistochemistry 815,815 cDNA ends) 119–123, 809–811 1021 prosthetics (foreign body 141 appearance 809 retroperitoneal schwannoma reactions) 3 radiation-induced tumors 3 genetics 130 654, 654 bone cement 911, 911 angiosarcoma 603, 604 histopathology 810–811, 810 reverse transcription PCR (RT- carbon fibers/graphite 911– mesothelioma 783 sarcomatoid 813 PCR) 119–123, 141, 977 912, 912 radiography 11, 29 immunohistochemistry 811, rhabdoid rhabdomyosarcoma metal fragments 912–913, 913 calciphylaxis 842 812–813 544–545, 545 polyethylene wear particles chondroma of soft parts 826 sarcomatoid 811 rhabdoid tumor, extrarenal 910, 911 cranial fasciitis 250 renal tumors see also 882–886 silicone 911, 912 fibrodysplasia ossificans 846 angiomyolipoma appearance 883, 883 protein kinase A (PRKAR1A mesenchymal glomus tumor 625 cytology 1007–1008, 1008 gene) 309–310, 315 chondrosarcoma 833 hereditary leiomyomatosis and differential diagnosis 758, 886, Proteus syndrome 272–273 myositis ossificans 12, 842, 843 renal cell cancer 445–446, 1008 gyriform lesions on the soles of neuroblastoma 871 461, 462–463 pseudo-rhabdoid tumors the feet 272 osteosarcoma 848 juxtaglomerular cell tumor 885 histopathology 272, 273 phosphaturic mesenchymal 633–634, 634 electron microscopy 883, 883 proto-oncogenes 115 see also tumor 835 renal capsule PEComa 520, 520 genetics 133, 882, 885–886 oncogenes pseudogout 840 synovial sarcoma 746 histopathology 883, 883 proximal-type epithelioid synovial chondromatosis 34, Wilms’ tumor 856, 882 history 882 sarcoma 758, 759 35, 828–829 reticulohistiocytoma 962–964, immunohistochemistry 883– psammoma bodies 663, 716, 792 TSGT 34 964 885, 884

1058

Index

rhabdomyoma 527–528 embryonal 534–535, 536 synovial sarcoma 57, 752, 752 imaging 20,31 adult 529, 529–530 and embryonic myogenesis SAF see superficial acral immunohistochemistry 56, cardiac 523 531, 534 fibromyxoma 73–74, 655 cytology 993, 993 pleomorphic 542, 543 salivary gland tumors 74 cellular 656 differential diagnosis 527–530, rhabdoid 545 SAM see superficial epithelioid 657, 658 993 rhabdomyoma-like 545 angiomyxoma gastrointestinal 661 fetal 528–529 sclerosing 536–537, 538 Sanger DNA sequencing 144 melanotic 664 genetics 529–530 spindle cell 536, 537 sarcoidosis, synovitis 907 malignant see malignant histopathology imaging 535, 539 sarcoma peripheral nerve sheath adult 529, 529 immunohistochemistry 49–52, epidemiology 2–3, 11, 975 tumor (MPNST) cardiac 523 51, 57, 546–547 etiology 3–4 malignant transformation fetal 528, 528 alveolar 546, 547 grading 4–5, 75–76 658–659 genital (vaginal) 495 ectomesenchymoma 545 histogenesis 2, 899–900 angiosarcoma 607, 659 juvenile 528, 528 embryonal 51, 53, 547 staging 5–6, 15–17 melanotic 661–664, 702 immunohistochemistry 50, fusion status markers 546–547 sarcoma botryoides (botryoid plexiform 656–657, 661 529 pleomorphic 543 rhabdomyosarcoma) with rosettes 657–658 fetal 528 spindle cell 538 533–534 vestibular 133, 654, 659–660 vaginal 493–494, 530 lipid-rich 544 histopathology 535, 537 schwannomatosis 654, 660 rhabdomyoma-like miRNA 117 sarcomatoid carcinoma schwannomin (merlin) 133, 659– rhabdomyosarcoma mixed alveolar and embryonal breast 805, 805 660 (RMS) 544, 545 544 lung 807, 808 sciatic nerve resection 1013 rhabdomyomatous mesenchymal oncogenesis 527 renal cell 811, 813 scintigraphy 11–12, 29 hamartoma 528–530, 530 pleomorphic 541–542, 994 chromophobe 813 sclerosing epithelioid rhabdomyosarcoma (RMS) 531– prognosis 547–548, 993 squamous cell 803, 803–804 fibrosarcoma 350–351 548 rhabdomyoma-like 544 laryngeal 804 as form of LGFMS 336, 350 alveolar 129, 134–135, 539– spindle cell/sclerosing 535–539 sarcomatoid mesothelioma 787– histopathology 351, 351 541, 878–879, 994 staging 547–548 788, 787–788 immunohistochemistry 74, anaplastic 543–544 treatment 531–532, 547–548, sarcomatous spindle cell GIST 351 anaplastic spindle cell 542 1018–1019 479, 479 sclerosing extramedullary appearance 531–532, 535 in adults 1031 SATB2 protein 74–75 myeloid tumor 424 chemotherapy 547–548, 1018– rheumatoid arthritis 904–905, 906 scalp angiosarcoma 602–603, sclerosing hemangioma see 1019, 1031 rheumatoid nodule (necrobiotic 603 benign fibrous classification 531, 993 granuloma) 905 scalp meningioma 714–715, 715 histiocytoma clear cell 544 rice bodies 905, 906 scapular tip tumors see sclerosing lipogranuloma 409, clinical features 531, 533–534, RMS see rhabdomyosarcoma elastofibroma 409 536, 539, 541–542 RNA scars (keloids) 201, 202 sclerosing liposarcoma 420–421, cytology 994, 995 extraction from formalin-fixed Schwann cells 637 421 alveolar 994, 994 specimens 140–141 schwannoma 654 see also sclerosing perineurioma 668– embryonal 993–994, 994 miRNA 116–117, 129, 136 neurofibromatosis type 2 670, 669 differential diagnosis 292, 527, in therapies 1032 ancient 655, 989–990 sclerosing rhabdomyosarcoma 535, 539, 541–542, 545, RNA-seq technique 123 appearance 654–655, 654 536–537, 538, 539 878–879, 994 ROS1 gene fusion 125, 127 gastrointestinal 660, 660 sclerotic fibroma of skin 211, 214 ectomesenchymoma 545–546, Rosai–Dorfman disease (RDD) melanotic 662, 662 sclerotic lipoma 395–396, 398 862–863 956–957 cellular 655–656 scrotal tumors embryonal 51, 108, 532, 533– histopathology 957, 958 cellular plexiform 657 adenomatoid 777 535, 993–994 immunohistochemistry 58, clinical feature 654, 656–657 aggressive angiomyxoma 498 epithelioid/rhabdoid 544–545 957, 959 conventional 654–655 angiomyofibroblastoma-like genetics 548 rosettes cytology 989, 989 tumor 496–497, 497 alveolar 129, 134–135, 539– giant hyaline rosettes in low- ancient schwannoma 990 leiomyoma 447–448, 449 541, 542, 878–879 grade fibromyxoid differential diagnosis 652, 656, liposarcoma 418 embryonal 108, 535 sarcoma 350 661, 664, 702, 990 melanotic neuroectodermal epithelioid 545 Homer Wright rosettes electron microscopy 655, tumor of infancy 709 mixed alveolar and neuroblastoma 867, 872, 874 663 necrotizing fasciitis (Fournier’s embryonal 544 PNETs 860, 860 epithelioid 657, 661 gangrene) 198 pleomorphic 542 schwannoma 657–658, 659 gastrointestinal 487, 660–661 paratesticular spindle cell and prognosis 547 round cell liposarcoma 24, 431 genetics 637, 661 rhabdomyosarcoma 535– spindle cell 538 cytology 981–982, 981 histopathology 655, 655 539 histopathology 532 histopathology 434 cellular 656, 657 serous papillary carcinoma alveolar 539, 540–541 RT-PCR (reverse transcription cystic 656 793, 793 alveolar leukemoid variant PCR) 119–123, 141, 977 gastrointestinal 660–661, smooth muscle hyperplasia 444 540 RTKs see tyrosine kinase receptors 661 SDH (succinate dehydrogenase) anaplastic 544 melanotic 662–663, 663–664 138, 724 botryoid 535, 537 S100 protein 56–58, 277 plexiform 657, 658 SDH-deficient tumors clear cell 544 melanoma, metastatic 56, 699, rosettes 659 GISTs 482–483, 483 ectomesenchymoma 863 701 hybrid tumors 660 treatment 488, 1025

1059

Index

SDH-deficient tumors (cont.) round cell tumor; Ewing spindle cell carcinoma paraganglioma 724–725 paraganglioma 724–725 sarcoma family of breast 805, 805 sunitinib 1025–1026 segmental neurofibromatosis tumors; neuroblastoma; squamous cell carcinoma 803– superficial acral fibromyxoma type 1 (NF1) 653 rhabdoid tumor, 804 (SAF) 310–313 seminoma 48, 817–818, 818 extrarenal spindle cell hemangioma 571– appearance 311 senile hemangioma (cherry SMARCA5-EWSR1 gene fusion 575 differential diagnosis 307, hemangioma) 575, 576 865 histopathology 573–575, 573– 312–313 septic arthritis 903–904, 904 SMARCB1/SMARCB1 see INI1/ 574 histopathology 311–312, 312 serous carcinoma 792–793, 792– INI1 spindle cell lipoma 391–394 immunohistochemistry 312, 793 smooth muscle actin (SMA) 50, cytology 978, 979 312 sex cord stromal tumors see 546 histopathology 393, 394–396 superficial angiomyxoma (SAM) granulosa cell tumor smooth muscle tumors 443 see pleomorphic variant 393– 306–310 SFT see solitary fibrous tumor also leiomyoma; 394, 397 clinical features 306–307, Shimada classification leiomyomatosis; pseudoangiomatoid variant 309 (neuroblastoma) 867– leiomyosarcoma 396 differential diagnosis 307–308 870, 873, 874 angiomyomatous hamartoma immunohistochemistry 394, genetics 309–310 Shulman syndrome (eosinophilic of lymph node 444, 445 397 histopathology 307, 308–309 fasciitis) 195–197, 198 hamartoma of skin 443–444, spindle cell liposarcoma 423, 423 superficial cervicovaginal signet ring cell-like schwannoma 444–445 spindle cell rhabdomyosarcoma myofibroblastoma 493 660 myxoid (uterine) 463 535–539 histopathology 493, 493 signet ring cells in breast scrotal smooth muscle histopathology 536, 537 immunohistochemistry 493, carcinoma 805 hyperplasia 444 immunohistochemistry 538 494 silicone of uncertain malignant spindle cell synovial sarcoma superficial plexiform breast implants potential 468–469 748–749, 749 schwannoma 657 ALCL 953 soft part chondroma see splenic angiosarcoma 606, 607 superficial vascular granuloma 409, 410 chondroma of soft parts split-fat sign 18, 20,30–31 pseudoaneurysm 580, 581 joint prostheses 911, 912 soft tissue tumors, definition and squamous cell carcinoma 758, surgery 1012–1021 simian virus 40 (SV40) 783 classification 1–2 803, 803–804 adjuvant/neoadjuvant single-strand conformation solitary circumscribed neuroma laryngeal 804 chemotherapy 1017– polymorphism (SSCP) see palisaded SS18-SSX gene fusion 123, 128, 1018, 1027–1029 assay 142 encapsulated neuroma 136, 753 adjuvant/neoadjuvant sinonasal hemangiopericytoma solitary epithelioid histiocytoma staging of tumors 5–6, 15–17 radiotherapy 1015–1016, (glomangiopericytoma) (reticulohistiocytoma) neuroblastoma 878 1020, 1031 631–632, 632 962–964, 964 rhabdomyosarcoma 547–548 amputation 1015 sinonasal leiomyoma 452 solitary fibrous tumor (SFT) STAT6-NAB2 gene fusion 330 desmoids 239–241, 1015 sinonasal meningioma 716–717, 324–330 stem cells (precursor cells) 2 DFSP 337, 1015 716 appearance 326, 326 sternocleidomastoid tumors see excisional margins 1013 sinus histiocytosis with massive clinical features 324–326, 330 branchial anlage mixed GISTs 487, 1020 lymphadenopathy see differential diagnosis 329–330, tumor palliative 1026 Rosai–Dorfman disease 793 sternomastoid tumor see liposarcoma 1014–1015, sinusoidal hemangioma 559 genetics 330 fibromatosis colli 1019–1020 sirolimus 518 giant cell angiofibroma variant Stewart–Treves syndrome for metastatic disease 1029 skeletal muscle tumors 527 see 330 (angiosarcoma in MPNST 1018 also myositis; histopathology 326–327, 327– lymphedematous myxofibrosarcoma 1018 rhabdomyoma; 328 extremities) 4, 603–604, palmar/plantar fibromatosis rhabdomyosarcoma giant cell angiofibroma 330, 604 234 classification 527 331 stomach GIST see gastric GIST preoperative work up 1012– neuromuscular choristoma malignant 328 stomach glomus tumor 624–626 1013 530, 530, 638, 638 uncertain potential 329 storage of tissue samples 8 reconstruction 1013–1014 rhabdomyomatous immunohistochemistry 70, strawberry nevus see infantile retroperitoneal sarcoma 1019– mesenchymal hamartoma 328–329, 329, 330 hemangioma 1021 528–530, 530 orbital 224, 324 stromatosis see endometrial surgical sponges, retained 934, skin grafts 1013 terminology 324, 777 stromal sarcoma 935 small cell carcinoma of the lung Sox10 (transcription factor) 58 STUMP (smooth muscle tumors surgical staging system (metastatic) 802–803, 806 SP3-EWSR1 gene fusion 865 of uncertain malignant (Enneking) 6, 18 immunohistochemistry 53–54, spectral karyotyping (SKY) 100 potential) 468–469 sustentacular cells 57, 723, 728 54, 806, 807 spider cells (cardiac subcutaneous lesions 20 SUZ12-JAZF1 gene fusion 123, intracranial 806 rhabdomyoma) 523 subungual tumors (glomus 503 small intestinal GISTs 476, 479 spina bifida 384 tumor) 624 sweat gland tumors appearance 480 spinal tumors see also paraspinal succinate dehydrogenase (SDH) eccrine acrospiroma 762, 763 histopathology 479–480, 481 tumors 138, 724 eccrine spiradenoma 762–763, in NF1 patients 483–484, 484 paraganglioma 738–740, 739 succinate dehydrogenase (SDH)- 764 small round blue cell tumors spinal cord-associated lipoma deficient tumors SWI/SNF complex see INI1/INI1 (SBRCT) 855–856 see 384–385 GISTs 482–483, 483 (SMARCB1/SMARCB1) also desmoplastic small spindle cell angiosarcoma 608 treatment 488, 1025 synaptophysin 53–54, 876

1060

Index

synovial chondromatosis 825, keratins 66–67, 750, 751 localized 916, 916 Touton-type giant cells 827–830 monophasic 52, 751 atypical and malignant in BFH 223, 988 appearance 827–828, 828 S100 57, 752, 752 921–922 in juvenile xanthogranuloma histopathology 828–830, 829 prognosis 745–746, 750 clinical features 915–916, 963 imaging 34, 35, 827, 828 visceral 746 918–920 TP53 see p53 (TP53) secondary 827, 829–830, 829 synovial villous hyperplasia cytology 987, 987 trabectedin 1030 synovial hemangioma 26–27 906–907, 907, 908 differential diagnosis 368, trabecular carcinoma of the skin synovial lipomatosis (lipoma systemic calcinosis see 921–922, 987 see Merkel cell carcinoma arborescens) 383–384, calciphylaxis diffuse extra-articular transcriptomics 123, 143–144, 383–384 919–920 977 synovial pathology (other than T-cell lymphomas diffuse intra-articular 918–919 transgelin 50 tumors) cutaneous ALCL 954 genetics 105, 914, 920–921 transitional cell carcinoma acute purulent synovitis (septic nasal-type NK/T-cell 952, 952 histopathology 811–813 arthritis) 903–904, 904 noncutaneous ALCL 952–954, atypical/malignant 922, 922 translocations 103–108, 117, 120 Baker (popliteal) cyst 17,31– 953 diffuse extra-articular 920, see also fusion genes 32, 33 panniculitis-like 950–952, 951 921 transplantation see biopsy 903 TAF15 protein 126 diffuse intra-articular 919, immunosuppressed foreign body reactions tamoxifen 1031–1032 920 patients bone cement 911, 911 target sign 30 localized type 916–917, 916– transport of specimens 92–93 carbon fibers/graphite 911– targeted therapies 137 918 traumatic neuroma 30, 639, 640 912, 912 for advanced disease 1030 localized type with TRE17 (USP6) gene fusion 125 metal fragments 912–913, for ALK-rearranged tumors 127 cartilaginous metaplasia tricarboxylic acid (TCA) cycle 913 for desmoids 241–242, 1032 919 dysfunction 138, 724–725 polyethylene wear particles for DFSP 129, 345–346 localized type infarction 918 trichodiscoma 307 910, 911 for ETV6-NTRK3 gene fusion imaging 21,34 trisomy 237, 246, 294, 506, 921 silicone 911, 912 tumors 128 immunohistochemistry 920 Triton tumor gout 841, 903, 907, 909–910 future directions 1027, 1032 localized 915–917 benign 530, 530, 638, 638 granulomatous synovitis 907– for GISTs 1027 testicular tumors malignant 683, 683 909 adjuvant therapy 1020, adenomatoid tumor 777 TRKs see tyrosine kinase mycobacterial 907–908, 909 1026–1027 seminoma 48, 817–818, 818 receptors in sarcoidosis 907 first-line therapy 132, 487– of the tunica vaginalis 783, TSGT see tenosynovial giant cell hemosiderotic synovitis 906– 488, 1024–1026 790–793, 793 tumor 907, 907–908 resistance 132, 486, 1020, TFE3/TFE3 TTF1 (thyroid transcription Lyme disease 904, 905 1024–1025, 1025, 1027 gene fusion 125 factor 1) 789, 805–806, osteoarthritis 905–906, 907 SDH-deficient tumors 488, TFE3-ASPSCR1 (ASPL) 129, 817 pseudogout 903, 910, 910 1025 900 tuberous sclerosis complex (TSC) tophaceous 838–839, 840– MDM2 inhibitors 131 TFE3-YAP1 599 513, 517–518, 523 841 mTOR pathway inhibitors as IHC marker 898 tuberous xanthoma 932 rheumatoid 904–905, 906 133, 518 TFG gene fusion 126 tubulopapillary mesothelioma synovial fluid analysis 903 for vestibular schwannoma 133 thalidomide 553 784–785, 784 synovial sarcoma 744–753 targetoid hemosiderotic thoracic paraganglioma 734 adenomatoid-like 785 appearance 746, 746 hemangioma (hobnail thorotrast contrast material 3, tumor migration 240 chemotherapy 1017–1018 hemangioma) 565–566, 934–935, 935 tumor necrosis factor (TNF) clinical features 744–746 567 thymolipoma 385–386, 385 1028 cytology 1003, 1003 taxanes 1018, 1030–1031 thymoma see ectopic tumor suppressor genes 116 differential diagnosis 18, 753, telangiectasia 554, 575 see also hamartomatous APC 137–138, 245–246 793, 1003 angiokeratoma thymoma (branchial FH 138 MPNST 680–684 port wine stain 575–576 anlage mixed tumor) INI1 (SMARCB1) 133–134, genetics 123, 128, 753 venous lake 577 thyroid carcinoma 184, 816–817, 758, 882, 885 histopathology 746–747 telomerase 877 817 NF1 108–110, 132–133, 637, biphasic 747–748, 747–748 tendinous xanthoma 932, 932 tibial tumors, adamantinoma 653 calcification 749 tendon pathology (other than 770, 770 NF2 133, 637, 659–660, 669 monophasic spindle cell tumors) tissue handling procedures 8 RB1 76, 116, 134 748–749, 749 amyloid 938 TKIs see tyrosine kinase SDH 138 poorly differentiated 750, ochronosis 913, 914 inhibitors TP53 see p53 (TP53) 750 rupture 914–915, 914 TLE1 (transducin-like enhancer tumoral calcinosis 837–838 hybrid with extraskeletal tendon sheath fibroma 209–211 of split 1) 752, 752 appearance 838, 838 myxoid chondrosarcoma appearance 213 TLS see FUS gene fusions histopathology 838, 839 750 histopathology 209–210, TMPRSS2 gene fusion 125 imaging 838, 838 imaging 18 213–214 TNM staging system 5 tunica vaginalis tumors 783, immunohistochemistry 750– tenosynovial giant cell tumor rhabdomyosarcoma 548 790–793, 793 753, 752, 1003 (TSGT) 914–922 tophaceous pseudogout 838–839 turban tumor 763 biphasic 68, 751 appearance histopathology 839, 841 Turner syndrome 562, 582 calretinin 72, 752 diffuse intra-articular 919, imaging 839, 840 “two-hit hypothesis” (Knudson) INI1 74 919 torticollis 251 108–109, 116, 137–138

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tyrosinase 58–60 urinary bladder leiomyoma 452 proliferations; Warburg effect 138 tyrosine hydroxylase 875, 876 urinary bladder paraganglioma telangiectasia well-differentiated liposarcoma tyrosine kinase inhibitors (TKIs) 729–734, 733–734 borderline see (WDLS) 416–424 137 urogenital solitary fibrous tumor hemangioendothelioma appearance 419, 419 crizotinib 127 325 endothelial biology 553–554 clinical features 417–419 in desmoids 241–242, 1032 uroplakin 2 (UP II) 811–813 endothelial cell markers 41–48 cytology 979–981, 980 in DFSP 129, 345–346 urothelial carcinoma 67–68, 811– genetic syndromes 587 differential diagnosis 381, 424 in GISTs 132 813 malignant see angiosarcoma; genetics 102, 131, 416–423 adjuvant 1020, 1026–1027 USP6 (TRE17) gene fusion 125 Kaposi’s sarcoma; venous histopathology 419 first-line therapy 132, uterine tumors see also leiomyosarcoma inflammatory 421–422, 421– 487–488, 1024–1026 endometrial stromal vasculogenesis 553–554 422 resistance 132, 486, 1020, sarcoma VEGF/VEGFR see vascular lipoma-like 419–420, 419 1024–1025, 1025, 1027 adenomatoid tumor 777, 778 endothelial growth myxoid 422, 422–423 imatinib see imatinib mesylate chemotherapy 1031 factor/receptor sclerosing 420–421, 421 lapatinib 133 epithelioid smooth muscle vena cava leiomyosarcoma 457, 458 spindle cell 423 midostaurin 128 tumors 463, 463 venous hemangioma 562, 563 imaging 22–24, 23 pazopanib 1030 inflammatory myofibroblastic venous lake 577 immunohistochemistry 423 regorafenib 1025–1026 tumor 289 venous leiomyosarcoma 457, 458, spindle cell 423 sunitinib 1025–1026 leiomyoma 72–73, 459–463 458 surgery 1014–1015 tyrosine kinase receptors (TKRs/ histopathology 461–462, 461 venous stasis, well-differentiated papillary RTKs) 127, 877 see also leiomyomatosis, intravenous acroangiodermatitis 580, mesothelioma 780–781, ALK/ALK (anaplastic 463–464, 464 580 781, 789–790, 791 lymphoma kinase); leiomyosarcoma 466–467, 467, Verocay bodies 643, 655, 989 whole-chromosome probes CD117 (KIT) 1031 verrucous hemangioma 560–561, (WCPs) 97 IGFR 136–137 malignant mixed müllerian 561 Wilms’ tumor 856, 881–882 MET 130, 136–137 tumor (carcinosarcoma) verruga peruana 579 Wnt signaling pathway 128, 138 NTRK3 127–128, 294 503–505, 504 vestibular schwannoma 133, 654, WT1/WT1 (Wilms’ tumor PDGFRA 132, 475, 1024 histopathology 504–505, 504 659–660 protein) PDGFRB 242 myxoid 463 vimentin 75 as an IHC marker 75, 76, 880 ROS1 125, 127 PEComa 520–521, 522 viruses, oncogenic 3 fusion with EWSR1 126–156, VEGFR 47, 553, 555, absent from IMT 291–292 881 593–594 vagal body paraganglioma 735 EBV 3–4, 467–468, 947, 949 WWTR-CAMTA1 gene fusion vaginal tumors herpes simplex 313 598–599 UICC-TNM staging system 5 cervicovaginal HHV8 3, 136, 612 ultra-deep targeted sequencing myofibroblastoma 493, HPV16 803 X chromosome inactivation, 145 493 Merkel cell polyomavirus 801– basis of clonality assay ultrasound (US) 14–15, 25, 32 mesodermal stromal polyp 802 139 ultrastructure see electron 491–493, 492 SV40 783 X-rays see radiography microscopy rhabdomyoma 493–494, 495, Von Hippel–Lindau disease type xanthogranuloma, juvenile see ultraviolet radiation 364, 798 530 2 (VHL-2) 725–726 juvenile umbilical polyp 251–253, 252 vanillylmandelic acid 870 von Recklinghausen’s disease see xanthogranuloma undifferentiated pleomorphic vascular endothelial growth neurofibromatosis type 1 xanthogranulomatous sarcoma (UPS) see factor/receptor (VEGF/ (NF1) inflammation 930–932, malignant fibrous VEGFR) 553 von Willebrand factor (vWF) 47, 931 histiocytoma (MFH) VEGFR2 555 610 xanthoma 226, 932 undifferentiated pleomorphic VEGFR3 47, 593–594 vulvar tumors disseminated 932 sarcoma with giant cells vascular malformations 554 angiomyofibroblastoma eruptive 932 see giant cell malignant vascular resection/reconstruction 494–496, 495, 496 planar (xanthelasma) 932 fibrous histiocytoma 1013 cellular angiofibroma 496–497, tendinous 932, 932 unknown primary 818–819 vascular tumors 497–498 tuberous 932 ureteral obstruction 200 benign see hemangioma; leiomyoma 459 urinary bladder inflammatory lymphangioma; papillary mesodermal stromal polyp YAP1-TFE3 gene fusion 599 myofibroblastic tumor endothelial hyperplasia; 491–493, 492 288–289, 292 reactive vascular PEComa 522 Zellballen 723

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