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The spectrum of rare morphological variants of cutaneous epithelioid angiosarcoma
Author(s) -
Wood Andrew,
Mentzel Thomas,
Gorp Joost,
Flucke Uta,
Huschka Ulrich,
Schneider Johann,
Bacchi Carlos E,
Calonje Eduardo,
Brenn Thomas
Publication year - 2015
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12589
Subject(s) - angiosarcoma , pathology , cd31 , medicine , context (archaeology) , cd68 , immunohistochemistry , hemangiosarcoma , epithelioid cell , sarcoma , biology , paleontology
Aims Unusual cytoplasmic alterations have recently been reported in poorly differentiated cutaneous angiosarcoma, making an accurate diagnosis challenging. As these tumours remain poorly documented, we aimed to study their clinicopathological characteristics more comprehensively. Methods and results Six cutaneous angiosarcomas with unusual cytoplasmic alterations were identified from referral files. All tumours arose as nodules or plaques (range: 05–195 mm) on sun‐damaged skin of the head and neck of elderly males (median age: 76.5 years). Histologically, the tumours were composed of enlarged epithelioid cells showing prominent signet ring ( n = 3), foam ( n = 2) or granular cell ( n = 1) change. Vasoformative elements were only focally noted. By immunohistochemistry, all tumours expressed CD 31 and avian v‐ets erythroblastosis virus E26 oncogene homologue ( ERG ). Foam cell change was associated with additional expression of CD 68 and CD 163. Follow‐up (median: 8 months) showed death from disease ( n = 1), death from a gastrointestinal bleed ( n = 1), and a cutaneous metastasis ( n = 1). Only two patients are alive with no evidence of disease. Conclusions Our findings outline the morphological spectrum of cytoplasmic change in cutaneous angiosarcoma. Awareness and a high degree of suspicion in the context of tumours affecting sun‐damaged skin of the elderly are necessary to direct appropriate immunohistochemical work‐up with inclusion of the endothelial cell markers CD 31 and ERG .