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Angiosarcomas

recurrence after surgical excision and radiother- Elisa Cinotti, Franco Rongioletti apy. In one case, the accompanying dense infl am- matory infi ltrate was attributable to a superimposed Cutaneous angiosarcoma is a rare, aggressive infection by Pseudomonas aeruginosa . vascular sarcoma that occurs in three main differ- Pathology: It is characterized by the same ent clinical settings: classic cutaneous angiosar- atypical vessels of the classical angiosarcoma, coma arising in sun-damaged skin of elderly with the addition of a prominent infl ammatory patients, cutaneous angiosarcoma associated lymphoid infi ltrate between the vessels, obliterat- with chronic lymphedema, and post radiation ing some or most of the channels (Fig. 2 ). The angiosarcoma. Recent studies have shown that infi ltrate can be diffuse or can be organized in high-level amplifi cation of MYC oncogene seems lymphoid follicles with germinal centers scat- to be specifi c for radiation and lymphedema- tered within the diffuse lymphoid infi ltrate. associated angiosarcoma. A new histological Vessels are poorly circumscribed, irregularly variant has been named pseudolymphomatous dilated, and anastomosing, lined by prominent, cutaneous angiosarcoma. In general, cutaneous atypical endothelial cells (Fig. 3 , 4) that usually angiosarcoma carries a poor prognosis, associ- express CD31 (Fig. 5), CD34, and D2-40. Most ated with 5-year overall survival rates between 10 of the cells of the lymphoid infi ltrate express and 30 %. strong immunoreactivity for CD3, CD4, CD5, Pseudolymphomatous angiosarcomas and CD45 markers, whereas only scattered cells Synonyms: Angiosarcoma with prominent express CD8. Most of the lymphocytes of the lymphocytic infi ltrate. germinal centers are positive for CD20, CD21, Introduction: Pseudolymphomatous cutane- CD79a, and Bcl-6 whereas Bcl-2 can be detected ous angiosarcoma, described by Requena et al . in in the cells of the mantle zone. 2007, is characterized by a prominent infl amma- Differential diagnosis: The main histological tory lymphoid infi ltrate that can mask the under- differential diagnoses are: (1) cutaneous B-cell lying vascular malignant proliferation and mimic follicle center lymphoma, (2) pseudolymphoma, a lymphomatous process. (3) Kimura's disease/angiolymphoid hyperplasia Clinical features: It presents with the same with eosinophilia (ALHE), and (4) Kaposi clinical features as the classical angiosarcoma, as sarcoma. a bruise-like areas or erythematous- violaceous Pseudolymphomatous angiosarcoma can nodule or plaque of the scalp, face and breast mimic both cutaneous lymphoma and pseudo- (Fig. 1 ). Three cases resembled facial rosacea, lymphoma, and the recognition of irregular anas- one case developed seven years after breast radio- tomosing vascular spaces lined by prominent therapy. Rongioletti el al. described a collision endothelial cells among the lymphocytic infi l- tumor with a basal cell carcinoma, and a transfor- trate allows the correct diagnosis. In particular, mation of an histologically- classical cutaneous among lymphomas the differential diagnosis is angiosarcoma in a pseudolymphomatous type in a mainly with cutaneous follicle center B-cell

F. Rongioletti et al. (eds.), Rare Malignant Skin Tumors, DOI 10.1007/978-1-4939-2023-5, 335 © Springer Science+Business Media New York 2015 336 Angiosarcomas

Fig. 1 Pseudolymphomatous cutaneous angiosarcoma. Fig. 4 Pseudolymphomatous cutaneous angiosarcoma An erythematous-violaceous plaque with bruise-like fea- The atypical cells are obscured by the dense lymphoid tures on the forehead infi ltrate

Fig. 2 Pseudolymphomatous cutaneous angiosarcoma. A Fig. 5 Pseudolymphomatous cutaneous angiosarcoma prominent infl ammatory lymphoid infi ltrate between the The atypical endothelial cells are outlined by CD31 vessels, obliterating some or most of the channels expression

lymphoma where the infi ltrate can be diffuse and/ or organized in follicles. The demonstration of a clonal rearrangement of heavy-chain immuno- globulin can help the diagnosis. Kimura's dis- ease/ALHE shows a more or less diffuse lymphoid infi ltration with lymphoid follicles and germinal centers in the presence of thick-walled blood vessels lined with “epithelioid” or “histio- cytoid” endothelial cells protruding into the lumen. The presence of eosinophils and the absence of atypical cytologic features of the endothelial cells are clues for Kimura's disease/ ALHE. A spindle-cell component and clusters of Fig. 3 Pseudolymphomatous cutaneous angiosarcoma At higher magnifi cation, vessels are poorly circumscribed, eosinophilic hyaline globules may help to distin- irregularly dilated, and anastomosing guish Kaposi sarcoma. Angiosarcomas 337

Prognosis: Cutaneous angiosarcoma is believed ous angiosarcoma: a rare variant of cutaneous angio- to have the worst prognosis quoad vitam among sarcoma readily mistaken for cutaneous lymphoma. Am J Dermatopathol. 2007;29:342–50. malignant skin tumors, with an overall 5-year sur- 3. Panizzon R, Schneider BV, Schnyder UW. Rosacea- vival rate ranging from 12% to 20% (median sur- like angiosarcoma of the face. Dermatologica. vival 18–28 months). Two cases series have showed 1990;181:252–4. a better prognosis of the pseudolymphomatous 4. Mentzel T, Kutzner H, Wollina U. Cutaneous angio- sarcoma of the face: clinicopathologic and immuno- variant with either longer survival or increased dis- histochemical study of a case resembling rosacea ease-free intervals, and less frequently metastases. clinically. J Am Acad Dermatol. 1998;38:837–40. However, further studies are necessary to confi rm 5. Rongioletti F, Albertini A-F, Fausti V, Cinotti E, the relatively better prognosis of pseudolymphoma- Parodi A, Fraitag S. Pseudolymphomatous cutane- ous angiosarcoma: a report of 2 new cases arising in tous variant of cutaneous angiosarcoma. an unusual setting. J Cutan Pathol. 2013;40: Treatment: Early wide surgical excision of the 848–54. tumour is the treatment of choice. The effi ciency 6. Diaz-Cascajo C, de la Vega M, Rey-Lopez A. Superinfected cutaneous angiosarcoma: a highly of adjuvant radiotherapy has not been established. malignant simulating an infl ammatory pro- cess. J Cutan Pathol. 1997;24:56–60. 7. Donghi D, Kerl K, Dummer R, Schoenewolf N, Cozzio A. Cutaneous angiosarcoma: own experience References over 13 years. Clinical features, disease course and immunohistochemical profi le. J Eur Acad Dermatol 1. Mentzel T. Sarcomas of the skin in the elderly. Clin Venereol. 2010;24:1230–4. Dermatol 2011;29:80-90. 8. Maddox JC, Evans HL. Angiosarcoma of skin and 2. Requena L, Santonja C, Stutz N, Kaddu S, Weenig soft tissue: a study of forty-four cases. Cancer. RH, Kutzner H, et al. Pseudolymphomatous cutane- 1981;15;48:1907–21. Index

A ES , 150 Acantholytic squamous cell carcinoma (ASCC) PH , 169 differential diagnosis , 4–5 Apocrine adenocarcinoma. See Primary cutaneous pathology , 3–4 apocrine adenocarcinoma prognosis , 5 , 63 pseudoacantholysis , 3, 5 Array comparative genomic hybridization (aCGH) , 234 vs. SCC , 3–5 ASC. See Adenosquamous carcinoma (ASC) treatment , 5 ASCC. See Acantholytic squamous cell carcinoma Adenoacanthoma of the sweat glands , 3 (ASCC) Adenosquamous carcinoma (ASC) ATLL. See Adult T-cell lymphoma/leukemia (ATLL) vs. ASCC , 4 Atrophic DFSP , 139 clinical features , 15 Atypical cellular blue nevus (ACBN) , 241 differential diagnosis , 16–17 Atypical fi broxanthoma (AFX) and mucoepidermoid carcinoma , 16 clinical features , 141–142 pathology , 15–16 differential diagnosis , 144–145 prognosis , 17 pathology , 143–144 treatment , 17 prognosis , 145 Adjuvant radiotherapy treatment , 145 CS , 24 Atypical intradermal smooth muscle neoplasm , 185, 186 DM , 208 Atypical spindle cell lipomatous tumor , 175, 176 fi brosarcoma , 135 MCC , 252 MPNST , 260 B PC , 55 Balloon cell malignant melanoma (BCMM) , 43 clinical features , 223 Adnexal CS , 23–25 differential diagnosis , 224–225 ADPC. See Digital papillary adenocarcinoma (ADPC) pathology , 223–224 Adult T-cell lymphoma/leukemia (ATLL) prognosis , 225 clinical features , 279, 280 treatment , 225 differential diagnosis , 280 Basal cell carcinoma (BCC) HTLV-1 infection , 279 FEP , 33 pathology , 279, 280 matrical differentiation prognosis , 280–281 clinical features , 37 T-cell non-Hodgkin lymphoma , 279 differential diagnosis , 38 treatment , 281 pathology , 37–38 AFX. See Atypical fi broxanthoma (AFX) prognosis , 38 Aggressive digital papillary adenocarcinoma , 103, 105 treatment , 38 Aggressive , 55 with a sarcomatous component , 24 Alveolar rhabdomyosarcoma , 189–191 SCC , 9 Amputation with shadow cells , 37, 38

F. Rongioletti et al. (eds.), Rare Malignant Skin Tumors, DOI 10.1007/978-1-4939-2023-5, 339 © Springer Science+Business Media New York 2015 340 Index

BCMM. See Balloon cell malignant melanoma (BCMM) clinical features , 7 Bednar tumor , 139 differential diagnosis , 9 Benign trichoblastoma , 42, 43 pathology , 7–9 Berti lymphoma , 291 prognosis , 9 Blastic plasmacytoid dendritic cell neoplasm (BPDCN) treatment , 9 clinical features , 307, 308 Colloid carcinoma , 115 pathology , 307, 308 Complete surgical excision. See also Surgical excision PDC , 307 CS , 24 prognosis , 308 DFSP , 140 treatment , 308 EHE , 159 Borderline melanoma (BM) , 227 FEP , 35 Borrelia infection , 284 malignant mixed tumor of the skin , 79 Bowen’s disease , 17 MEC , 21 BPDCN. See Blastic plasmacytoid dendritic cell myxoid LPS , 179–180 neoplasm (BPDCN) PC , 110 Breast carcinoma pilomatrix carcinoma , 55 clinical features , 313, 314 SCC , 9 differential diagnosis , 314, 316 SEC , 101 pathology , 313–315 trichoblastoma , 43 prognosis , 316 Composite hemangioendothelioma (CHE) treatment , 316 clinical features , 155 Brooke-Spiegler syndrome , 85 differential diagnosis , 155 pathology , 155 prognosis , 156 C treatment , 156 Calcifying epitheliocarcinoma. See Pilomatrix vascular tumor , 155 carcinoma (PC) Congenital-infantile fi brosarcoma , 134, 135 Carcinoma sebaceum adenomatoides , 57 Conventional melanocytic marker , 212 Carcinosarcoma of skin (CS) CS. See Carcinosarcoma of skin (CS) clinical features , 23 Cutaneous g/δ T-cell lymphomas (CGD-TC) differential diagnosis , 24 clinical features , 295, 296 pathology , 23–24 Differential Diagnosis , 296 prognosis , 24 pathology , 295, 296 treatment , 24, 26 prognosis , 296 CCS. See Clear cell sarcoma (CCS) treatment , 296–297 CD8+ aggressive epidermotropic cytotoxic T-cell Cutaneous leiomyosarcoma lymphoma (CD8+ AECTCL) clinical features , 185 clinical features , 291, 292 differential diagnosis , 186–187 differential diagnosis , 291–292 pathology , 185–186 pathology , 291, 292 prognosis , 187 prognosis , 292 treatment , 187 treatment , 293 Cutaneous metastases Ceruminous carcinoma , 63 breast carcinoma (see Breast carcinoma) CGD-TC. See Cutaneous g/δ T-cell lymphomas gastrointestinal tract (CGD-TC) clinical features , 317–318 Chalazodermic Besnier-Boeck-Schaumann's disease , 267 differential diagnosis , 320 CHE. See Composite hemangioendothelioma (CHE) pathology , 318–320 Chordoma , 217 prognosis , 320 Classic-type ES , 147, 148 treatment , 320 Clear-cell , 81 of internal malignancies , 173–174 Clear-cell , 81 kidney Clear cell sarcoma (CCS) chemotherapeutic treatments , 327 clinical features , 243 clinical features , 325, 326 differential diagnosis , 244, 245 differential diagnosis , 326–327 pathology , 243, 244 pathology , 325–327 prognosis , 245 prognosis , 327 treatment , 245 lung cancer (see Lung cancer, cutaneous metastases) Clear cell SCC urogenital system , 327–328 vs. clear cell BCC , 9 Cylindrocarcinoma. See Malignant (MC) Index 341

D clinical features , 119 Dabska’s tumor , 165 differential diagnosis , 120 Dermal leiomyosarcoma. See Superfi cial (dermal) pathology , 119 leiomyosarcoma prognosis , 120 Dermatofi broma , 139 treatment , 120 Dermatofi brosarcoma protuberans (DFSP) ENTL. See Extranodal NK/T-cell lymphoma (ENTL) clinical features , 137–138 Epidermal-derived CS , 23, 24 COL1A1–PDGFB translocation , 137, 139 Epidermoid carcinoma of the skin with adnexal differential diagnosis , 139–140 metaplasia, 11 molecular studies , 139 Epithelial membrane antigen (EMA) pathology , 138–139 balloon cell melanoma , 224 prognosis , 140 merkel cell carcinoma , 251 treatment , 140 myxoid melanoma , 217 Desmoplastic benign , 50 Epithelioid angiosarcoma , 5 Desmoplastic melanoma (DM) Epithelioid hemangioendothelioma (EHE) clinical features , 205, 206 clinical features , 157 differential diagnosis , 207–208 differential diagnosis , 158–159 pathology , 205–207 pathology , 157–158 prognosis , 208 prognosis , 159 treatment , 208 treatment , 159 DFSP. See Dermatofi brosarcoma protuberans (DFSP) Epithelioid sarcoma (ES) Diffuse large B-cell lymphoma leg type (DLBL-LT) clinical features , 147 clinical features , 299, 300 differential diagnosis , 149 differential diagnosis , 299–300 immunohistochemistry , 149 pathology , 299, 300 molecular studies , 149 PCFCL , 299 pathology , 148–149 prognosis , 300 prognosis , 149–150 treatment , 300 treatment , 150 Digital papillary adenocarcinoma (ADPC) Epithelioid sarcoma-like hemangioendothelioma , 169 clinical features , 103 Epithelioma spinocellular segregans , 5 cytologic atypia , 103, 104 Epstein-Barr virus (EBV) , 287 differential diagnosis , 105 ES. See Epithelioid sarcoma (ES) pathology , 103–105 Evans' tumor. See Low-grade fi bromyxoid sarcoma prognosis , 105 (LGFMS) treatment , 105 Extramammary Paget's disease (EMPD) DLBL-LT. See Diffuse large B-cell lymphoma leg type clinical features , 69–70 (DLBL-LT) differential diagnosis , 71–72 DM. See Desmoplastic melanoma (DM) histochemistry , 70–71 immunohistochemistry , 71 vs. pagetoid Bowen’s disease , 72 E pathology , 70 Early wide surgical excision , 5 primary form , 69 hidradenocarcinoma , 82 prognosis , 72 MRT , 195 secondary form , 69 PMCS , 116 Toker cell hyperplasia , 72 Eccrine adenocarcinoma , 4 treatment , 72 Eccrine porocarcinoma , 107 Extranodal NK/T-cell lymphoma (ENTL) Eccrine spiradenocarcinoma , 89 clinical features , 287, 288 Eccrine syringofi broadenoma (ESFA) , 34 differential diagnosis , 288 Eccrine syringomatous carcinoma. See Syringoid EBV , 287 eccrine carcinoma (SEC) pathology , 287, 288 EHE. See Epithelioid hemangioendothelioma (EHE) prognosis , 288–289 Embryonal rhabdomyosarcoma , 189–191 treatment , 289 EMPD. See Extramammary Paget's disease (EMPD) Extraocular SC , 57, 60 EMPSGC. See Endocrine mucin-producing sweat Extrarenal rhabdoid tumour , 193 gland carcinoma (EMPSGC) Extraskeletal myxoid chondrosarcoma , Endocrine mucin-producing carcinoma 132, 216 (EMPSGC) Extraskeletal osteosarcoma (EO) , 199 342 Index

F Intravascular lymphoma (IVL) FEP. See Fibroepithelioma of Pinkus (FEP) capillaries and venules , 303 Fibroepithelial type of basal-cell carcinoma , 33 clinical features , 303 Fibroepithelioma of Pinkus (FEP) differential diagnosis , 303–304 clinical features , 33 pathology , 303, 304 differential diagnosis , 34 prognosis , 304 pathology , 33–34 treatment , 304 prognosis , 35 treatment , 35 Fibrosarcoma K clinical features , 133 Kaposiform hemangioendothelioma (KHE) vs. DFSP , 140 clinical features , 161 differential diagnosis , 134–135 differential diagnosis , 162–163 pathology , 133–134 pathology , 161–162 prognosis , 135 prognosis , 163 treatment , 135 treatment , 163 Fibrosarcomatous DFSP , 139 Kasabach-Merritt syndrome (KMS) , 161, 163 Folliculotropic mycosis fungoides (FMF) Keratinizing tumors , 7 clinical features , 263, 264 KHE. See Kaposiform hemangioendothelioma (KHE) differential diagnosis , 266 Kidney, cutaneous metastases pathology , 265–266 chemotherapeutic treatments , 327 prognosis , 266 clinical features , 325, 326 treatment , 266 differential diagnosis , 326–327 pathology , 325–327 prognosis , 327 G Granulomatous slack skin (GSS) clinical features , 267, 268 L differential diagnosis , 269–270 Laryngeal carcinoma , 332 medical history , 267 Leiomyosarcoma , 132 pathology , 267–269 LELCS. See Lymphoepithelioma-like carcinoma of the prognosis , 270 skin (LELCS) treatment , 270 LGFMS. See Low-grade fi bromyxoid sarcoma (LGFMS) Liposarcoma , 132, 216 Localized pagetoid reticulosis (LPR) H clinical features , 271, 272 Hemangioendothelioma , 212 . See also Kaposiform differential diagnosis , 272 hemangioendothelioma (KHE) pathology , 271–272 Hemangioma with Kaposi's sarcoma-like features. prognosis , 272 See Kaposiform hemangioendothelioma treatment , 273 (KHE) Locally aggressive adnexal carcinoma , 97 Hemophagocytic syndrome (HPS) , 283 Low-grade dedifferentiated liposarcoma , 176 Hereditary cutaneous leiomyomatosis and renal cell Low-grade fi bromyxoid sarcoma (LGFMS) , 131 cancer (HLRCC), 185 clinical features , 125 Hidradenocarcinoma differential diagnosis , 126–127 clinical features , 81 pathology , 125–126 differential diagnosis , 81–82 prognosis , 127 pathology , 81 treatment , 127 prognosis , 82 LPR. See Localized pagetoid reticulosis (LPR) Histiocytoid carcinoma of the eyelid. Lung cancer, cutaneous metastases See Primary signet-ring cell/histiocytoid clinical features , 321, 322 carcinoma of eyelid differential diagnosis , 322 Hobnail hemangioendothelioma , 165 pathology , 321, 322 Hodgkin disease , 270 prognosis , 322 Hyalinizing spindle cell tumor with giant rosettes , 126 treatment , 323 Lymphoepithelioma-like carcinoma of the skin (LELCS) clinical features , 27 I differential diagnosis , 27–28 Infl ammatory fi brosarcoma , 131, 135 pathology , 27 International Melanoma Pathology Study Group , 231 prognosis , 28 Intraepidermal melanocytes , 228 treatment , 29 Index 343

M pathology , 193–194 MAC. See Microcystic adnexal carcinoma (MAC) prognosis , 195 Malignant blue nevus (MBN) treatment , 195 clinical features , 239, 240 Malignant , 97, 99 differential diagnosis , 240–241 Malignant trichoblastoma , 41 nevus-like or associated melanoma , 239 Matrical carcinoma , 38, 54 pathology , 239–241 Matricarcinoma , 54 prognosis , 241 MBN. See Malignant blue nevus (MBN) treatment , 241 MC. See Malignant cylindroma (MC) Malignant chondroid syringoma , 77 MCC. See Merkel cell carcinoma (MCC) Malignant clear-cell , 81 MDM. See Minimal deviation melanoma (MDM) Malignant cylindroma (MC) MEC. See Mucoepidermoid carcinoma (MEC) clinical features , 85 Meibomian gland carcinoma , 57 differential diagnosis , 86 Melanocytic tumors of uncertain malignant potential pathology , 85–86 (MELTUMP) , 241 prognosis , 86 Merkel cell carcinoma (MCC), 123 treatment , 86 clinical features , 249–250 Malignant , 85 differential diagnosis , 251–252 Malignant eccrine , 81 medical history , 249 Malignant eccrine (MES) pathology , 250–251 clinical features , 89 porocarcinoma , 110 differential diagnosis , 91 prognosis , 252 immunohistochemistry , 90 treatment , 252 pathology , 89–90 MES. See Malignant eccrine spiradenoma (MES) prognosis , 91 Metaplastic ossifi cation , 201 treatment , 91 Metastatic rhabdoid melanoma , 220 Malignant extrarenal rhabdoid tumour , 193 MFS. See Myxofi brosarcoma (MFS) Malignant fi brous histiocytoma , 140 Microcystic adnexal carcinoma (MAC) Malignant fi brous histiocytoma (MFH) , 141 clinical features , 95 Malignant mixed tumor of skin differential diagnosis , 97 clinical features , 77 immunohistochemistry , 97 differential diagnosis , 79 pathology , 95–97 pathology , 77–79 prognosis , 97 prognosis , 79 treatment , 98 treatment , 79 Minimal deviation melanoma (MDM) Malignant myxoid melanoma (MMM) clinical features , 227, 228 clinical features , 215 differential diagnosis , 228–229 differential diagnosis , 216–217 pathology , 227–228 pathology , 215, 216 prognosis , 229 prognosis , 217 treatment , 229 treatment , 217 Mixed adenocarcinoma and epidermoid carcinoma , 16 Malignant nodular clear-cell hidradenoma , 81 Mixed adenocarcinoma and squamous cell carcinoma , 16 Malignant peripheral nerve sheath tumor (MPNST) MMM. See Malignant myxoid melanoma (MMM) clinical features , 257–258 Mohs micrographic surgery vs. DFSP , 140 ASC , 17 differential diagnosis , 260 cutaneous leiomyosarcoma , 187 NF-1 , 257 DFSP , 140 pathology , 257–260 EMPD , 72 prognosis , 260 kidney , 327 treatment , 260 LELCS , 29 , 54 MAC , 98 Malignant pilomatrixoma , 54 MEC , 21 Malignant proliferating trichilemmal tumor (MPTT) PC , 110 clinical features , 45 pilomatrix carcinoma , 55 differential diagnosis , 46 PMCS , 116 pathology , 45–46 primary adenoid cystic carcinoma , 75 prognosis , 46 primary cutaneous apocrine adenocarcinoma , 65 treatment , 47 primary signet-ring cell/histiocytoid carcinoma of Malignant rhabdoid tumor (MRT) eyelid , 113 clinical features , 193 SC , 60 differential diagnosis , 194–195 SEC , 101 344 Index

MPNST. See Malignant peripheral nerve sheath tumor O (MPNST) Ocular SC , 57, 60 MPTT. See Malignant proliferating trichilemmal tumor Osteosarcoma , 199 (MPTT) M R T . See Malignant rhabdoid tumor (MRT) Mucinous adenocystic carcinoma , 116 P Mucinous carcinoma , 119 Paget disease , 12 Mucinous sweat gland adenocarcinoma , 116 Pagetoid Bowen’s disease , 72 Mucin-secreting adenocarcinomas , 212 PC. See Pilomatrix carcinoma (PC); Porocarcinoma (PC) Mucoepidermoid carcinoma (MEC) PC-EO. See Primary cutaneous extraskeletal vs. ASC , 19, 20 osteosarcoma (PC-EO) clinical features , 19–20 Perinuclear eosinophilic hyaline globules , 219 differential diagnosis , 20–21 Periocular SC , 57 pathology , 20 Periodic acid-Schiff (PAS) prognosis , 21 BCMM , 224 treatment , 21 ES/PNET , 253 Muir–Torre syndrome , 57–59 malignant myxoid melanoma , 215 Multinucleated giant balloon cells , 225 PH. See Pseudomyogenic hemangioendothelioma (PH) Mycosis fungoides. See Folliculotropic mycosis Pigmented DFSP , 139 fungoides (FMF) Pilomatrical carcinoma , 38 Myelodysplastic syndrome , 307 Pilomatricoma , 38 Myoepithelial cells , 119 Pilomatrix carcinoma (PC) Myxofi brosarcoma (MFS) basaloid cells , 53–55 clinical features , 129 clinical features , 53 differential diagnosis , 131–132 differential diagnosis , 54–55 pathology , 129–131 pathology , 53–54 prognosis , 132 prognosis , 55 treatment , 132 treatment , 55 Myxoid DFSP , 131, 139 Pinkus epithelioma. See Fibroepithelioma of Pinkus Myxoid liposarcoma (LPS) (FEP) clinical features , 179–180 Pinkus tumor. See Fibroepithelioma of Pinkus (FEP) differential diagnosis , 181 Pityriasis lichenoides et varioliformis acuta immunohistochemistry , 181 (PLEVA) , 292 molecular studies , 181 Plaque-like CD34-positive dermal fi broma , 140 pathology , 180–181 Pleomorphic hyalinizing angiectatic tumor (PHAT) , 131 prognosis , 181 Pleomorphic rhabdomyosarcoma , 189–191 treatment , 181–182 Pleomorphic tumors , 7 PMCS. See Primary mucinous carcinoma of the skin (PMCS) N Porocarcinoma (PC) Nasopharyngeal lymphoepithelioma , 28 clinical features , 107 Nested melanoma of the elderly (NME) differential diagnosis , 109–110 clinical features , 237, 238 histochemistry , 109 pathology , 237, 238 immunohistochemistry , 109 prognosis , 237 pathology , 107–109 treatment , 237 prognosis , 110 Neuroendocrine carcinoma , 119 treatment , 110 Neurofi broma , 140 Premalignant fi broepithelial tumor of the skin , 33 Neurofi bromatosis type 1 (NF-1) , 257 Primary cutaneous adenoid cystic carcinoma Neurotropism , 208 clinical features , 73 Nevoid melanoma (NM) differential diagnosis , 74 clinical features , 227, 228 pathology , 73–74 differential diagnosis , 228–229 prognosis , 75 pathology , 227–228 treatment , 75 prognosis , 229 Primary cutaneous apocrine adenocarcinoma treatment , 229 clinical features , 63 NME. See Nested melanoma of the elderly (NME) differential diagnosis , 65 Nodular hidradenocarcinoma , 81 histochemistry , 65 Nonkeratinizing tumors, 7 immunohistochemistry , 65–66 Index 345

vs. metastatic apocrine carcinoma , 65 RH. See Retiform hemangioendothelioma (RH) pathology , 63–65 Rhabdoid melanoma prognosis , 65 clinical features , 219 treatment , 65 differential diagnosis , 221 Primary cutaneous Ewing sarcoma/primitive pathology , 219–221 neuroectodermal tumor , 123 prognosis , 221 Primary cutaneous extraskeletal osteosarcoma (PC-EO) treatment , 221 clinical features , 199 Rhabdomyosarcoma , 217 differential diagnosis , 200–201 Round cell liposarcoma , 179–181 pathology , 199–200 prognosis , 201 treatment , 201 S Primary cutaneous follicle center lymphoma Sarcoma metastases , 332 (PCFCL) , 299 Sarcomatoid carcinoma , 23 Primary cutaneous MEC , 19 SC. See (SC) Primary cutaneous rhabdomyosarcoma (PC-RMS) SCC. See Squamous cell carcinoma (SCC) clinical features , 189 SCCMM. See Squamous cell carcinoma with mucinous differential diagnosis , 191–192 metaplasia (SCCMM) head and neck , 189 Schwann cells , 123 pathology , 189–191 SCL. See Spindle cell liposarcoma (SCL) pediatrics , 192 Sclerosing epithelioid fi brosarcoma (SEF) , 134 prognosis , 192 Sclerosing sweat duct (syringomatous) carcinoma , 99 skin neoplasms , 189 Sebaceous carcinoma (SC) treatment , 192 clinical features , 57 Primary EMPD , 69, 71, 72 differential diagnosis , 59 Primary metaplastic carcinoma , 23 incidence , 57 Primary mucinous carcinoma of the skin (PMCS) pathology , 58–59 clinical features , 115 prognosis , 59 differential diagnosis , 116 treatment , 59–60 pathology , 115 Seborrheic keratosis (SK) , 33, 34 prognosis , 116 SEC. See Syringoid eccrine carcinoma (SEC) treatment , 116–117 Secondary EMPD , 69, 71, 72 Primary signet-ring cell/histiocytoid carcinoma SEF. See Sclerosing epithelioid fi brosarcoma (SEF) of eyelid Sentinel lymph node biopsy clinical features , 111 clear cell sarcoma , 245 differential diagnosis , 111, 113 ES , 150 pathology , 111 hidradenocarcinoma , 82 prognosis , 113 MES , 91 treatment , 113 minimal deviation melanoma , 229 Progressive and recurrent dermatofi broma , 139 nevoid melanoma , 229 Proliferating pilomatricoma , 55 PC , 110 Proliferating trichilemmal tumor , 50 primary adenoid cystic carcinoma , 75 Proximal-type ES , 147, 148 primary cutaneous apocrine adenocarcinoma , 65 Pruritus , 275 SC , 60 Pseudoglandular squamous cell carcinoma , 3–5 signet-ring cell melanoma , 213 Pseudomyogenic hemangioendothelioma (PH) spitzoid melanoma , 235 clinical features , 169 Signet-ring cell melanoma differential diagnosis , 170–171 clinical presentation , 211 pathology , 169–170 conventional-appearing melanoma , 211, 212 prognosis , 171 differential diagnosis , 212, 213 treatment , 171 immunohistochemical staining , 211 intracytoplasmic vacuolization , 211, 212 metastatic or recurrent lesions , 211 R prognosis , 213 Renal cell carcinoma marker (RCC-Ma) , 326 treatment , 213 Retiform hemangioendothelioma (RH) Signet-ring cell SCC clinical features , 165 clinical features , 7 differential diagnosis , 166–167 differential diagnosis , 9 pathology , 165–166 pathology , 7–9 prognosis , 167 prognosis , 9 treatment , 167 treatment , 9 346 Index

Skin metastases , 173 pathology , 185–186 SMF. See Syringotropic mycosis fungoides (SMF) prognosis , 187 Solid papillary carcinoma , 119 treatment , 187 Spindle cell liposarcoma (SCL) Surgical excision clinical features , 175 balloon cell melanoma , 225 differential diagnosis , 176–177 breast carcinoma , 316 pathology , 175–176 desmoplastic melanoma , 208 prognosis , 177 DLBL-LT , 300 treatment , 177 ES/PNET , 255 Spitz nevus , 231 fi brosarcoma , 135 Spitzoid melanoma gastrointestinal tract , 320 clinical features , 231, 232 GSS , 270 differential diagnosis , 234 kidney , 327 pathology LGFMS , 127 aCGH , 234 localized pagetoid reticulosis , 273 asymmetrical dermal-based tumor , 233 malignant blue nevus , 241 CD99 , 234 MCC , 262 cytologic atypia , 233 MPNSTs , 260 dermal component , 231–232 PH , 169 HMB45 , 233 primary adenoid cystic carcinoma , 75 junctional nests , 231, 232 RH , 167 molecular diagnostic studies , 234 SCCMM , 13 prognosis , 234 SCL , 177 treatment , 234–235 Sweat gland carcinoma , 107 SPTCL. See Subcutaneous panniculitis-like T-cell Sweat gland carcinoma with syringomatous features , 95 lymphoma (SPTCL) Syringoid eccrine carcinoma (SEC) Squamoid eccrine ductal carcinoma , 99 clinical features , 99 Squamous cell carcinoma (SCC) differential diagnosis , 101 clear cell (See Clear cell SCC) pathology , 99–100 with heterologous mesenchymal differentiation , prognosis , 101 23, 24 treatment , 101 mucinous metaplasia (See Squamous cell carcinoma Syringotropic mycosis fungoides (SMF) with mucinous metaplasia (SCCMM)) clinical features , 275, 276 signet-ring cell (See Signet-ring cell SCC) differential diagnosis , 276 Squamous cell carcinoma with mucinous metaplasia pathology , 275, 276 (SCCMM) prognosis , 276 clinical features , 11 treatment , 276 differential diagnosis , 12 mucinous cells , 11–12 pathology , 11–12 T prognosis , 12 TC. See (TC) treatment , 13 T-cell non-Hodgkin lymphoma , 279 Storiform neurofi broma , 140 Thyroid gland tumors , 331–332 Subcutaneous leiomyosarcoma Toker cell hyperplasia , 72 clinical features , 185 Trichilemmal carcinoma (TC) pathology , 185 clinical features , 49 prognosis , 187 differential diagnosis , 50 treatment , 187 pathology , 49–50 Subcutaneous panniculitis-like T-cell lymphoma prognosis , 51 (SPTCL) treatment , 51 clinical features , 283 Trichoblastic carcinoma differential diagnosis , 283–285 vs. basal cell carcinoma , 42–43 GD-TCL , 283 clinical features , 41 pathology , 283, 284 differential diagnosis , 42–43 prognosis , 284 pathology , 42 T-cell receptor (TCR) , 283 prognosis , 43 treatment , 284 treatment , 43 Superfi cial (dermal) leiomyosarcoma Trichomatrical carcinoma , 54 clinical features , 185 Tumor of follicular infundibulum (TFI) , 34 differential diagnosis , 186–187 Tyrosine kinase inhibitor therapy , 43 Index 347

U Wide surgical excision , 29, 47, 65, 86 . See also Surgical Undifferentiated carcinoma with lymphoid stroma , 27 excision Undifferentiated pleomorphic sarcoma (UPS) , 141 MFS , 132 Urogenital system , 327–328 Woringer-Kolopp type mycosis fungoides. See Localized pagetoid reticulosis (LPR)

W Wegener's granulomatosis. , 269 Z Well-differentiated liposarcomas , 175, 176 Zosteriform/herpetiform metastasis , 313