Concomitant renal cell carcinoma and metastatic epithelioid angiosarcoma with microangiopathy

Epithelioid angiosarcoma is an extremely rare clinical entity. Recognized only in recent years, epithelioid angiosarcoma mimicks epithelial tumors, both morphologically and immunohistochemically. It is very aggressive, assuming a rapid, metastatic and fatal course. This is a report of a case with an unequivocal diagnosis of epithelioid angiosarcoma and concomitant renal cell carcinoma. Reports of cancer with double origins of this combination, in patients without inherited von Hippel-Lindau disease, are extremely rare in the English literature. A review of the literature encompassing all cases of epithelioid angiosarcoma since 1983 is included.