Primary epithelioid angiosarcoma of the adrenal gland - Case report and review of the literature

Primary angiosarcoma of the adrenal gland is extremely rare. Here, we repor t on a 70-year-old man with an anglosarcoma of the right adrenal gland who died 3 weeks after tumor resection due to intestinal infarction and acute r enal failure. No metastases were found at autopsy. Histologically, the tumo r showed a predominantly epithelioid differentiation. Immunohistochemical e xamination revealed positive reactivity for cytokeratin, epithelial membran e antigen, vimentin, factor VIII-related antigen, CD31, CD34 and Ulex europ aeus agglutinin-1. Features of endothelial origin were also demonstrable by electron microscopy. The differential diagnosis of this uncommon neoplasm is discussed. The present case emphasizes problems in differential diagnosi s that arise from its epithelioid differentiation. A review of the literatu re underlines the poor clinical outcome of adrenal anglosarcoma.