title: The spectrum of rare morphological variants of cutaneous epithelioid angiosarcoma.
abstract: OBJECTIVE
  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.
  
  
  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 CD31 and avian v-ets erythroblastosis virus E26 oncogene homologue (ERG). Foam cell change was associated with additional expression of CD68 and CD163. 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 CD31 and ERG.
authors.affiliation: Department of Pathology, Western General Hospital and The University of Edinburgh, Edinburgh, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Wood
authors.forename: Thomas
authors.initials: T
authors.lastname: Mentzel
authors.forename: Joost
authors.initials: J
authors.lastname: van Gorp
authors.forename: Uta
authors.initials: U
authors.lastname: Flucke
authors.forename: Ulrich
authors.initials: U
authors.lastname: Huschka
authors.forename: Johann
authors.initials: J
authors.lastname: Schneider
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Bacchi
authors.forename: Eduardo
authors.initials: E
authors.lastname: Calonje
authors.forename: Thomas
authors.initials: T
authors.lastname: Brenn
mesh_terms: D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D018323:Hemangioendothelioma, Epithelioid / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D012878:Skin Neoplasms / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: angiosarcoma; immunohistochemistry; outcome; signet ring cell; skin