[Primary renal epithelioid angiosarcoma with transitional cell carcinoma in renal pelvis]

To understand the clinical characteristics of primary renal epithelioid angiosarcoma. We used clinical pathology, immunohistochemistry and electron microscopy. Analysis and discussion about the case were combined with a review of the literature. We first report a female case of the left renal epithelioid angiosarcoma and concomitantly, transitional cell carcinoma developing from the renal pelvis at age 69. Epithelioid angiosarcoma showed positive results for FVIIIRA+, UEA, CD31, Cytokeratin and EMA. Weibel-Palade bodies were identified by electron microscopy. Primary renal epithelioid angiosarcoma was a rare clinical entity with a poor prognosis, which mimics epithelial tumor, both morphologically and immunohistochemically, and may be lead to misdiagnosis. The combined use of endothelial cell markers including FVIIIRA, CD31, UEA and electronmicroscopy can confirm the diagnosis of this neoplasm.