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RESIDENT’S CORNER

Epithelioid PEComa (epithelioid angiomyolipoma) of the kidney: a rare tumor subtype for patients presenting with an enhancing renal mass

Adam B. Shrewsberry¹, Gabriel L. Sica², Adeboye O. Osunkoya^1,2, Daniel J. Canter¹

1 Department of Urology, Emory University School of Medicine, Atlanta, Georgia, USA
2 Department of Pathology, Emory University School of Medicine, Atlanta, Georgia, USA
Address correspondence to Dr. Daniel J. Canter, Department of Urology, Emory University, 1365 Clifton Road, Clinic B, Suite 1400, Atlanta, GA30322 USA

Canadian Journal of Urology 2013, 20(1), 6643-6645.

Abstract

Epithelioid angiomyolipomas, or perivascular epithelioid cell tumors (epithelioid PEComas) of the kidney, are histologically related to renal angiomyolipomas (AMLs). However, in contrast to typical AMLs, this rare tumor can exhibit an aggressive clinical course with approximately 50% of reported cases demonstrating disease progression. In this report, we present a case of a 24-year-old female with a history of stone disease who was incidentally found to have a 9.0 cm right renal mass that was difficult to characterize radiographically preoperatively. The patient underwent a right radical nephrectomy, and pathology revealed a renal epithelioid PEComa.

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Keywords

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