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  • Clinical

    • age: elderly
    • site: lower extremities (40%), arm, trunk, head/neck, abdominal cavity
    • associations: lymphedema, coagulopathy, anemia
  • Histology

    • vessels/clefts lined by tumor cells, solid areas
    • lining: can be single layer or has projections
    • extensive hemorrhage
    • cytology: mixture of epithelioid and spindled, high grade, mitosis
    • epithelioid angiosarcoma
      • this variant is composed entirely of epithelioid cells
      • mimicks carcinoma in appearance
  • Stains

    • positive: CD31 (most sensitive and good specificity), CD34, VWF (most specific)
    • 1/3 express CK
    • negative: HHV8