Renal epithelioid angiomyolipoma presenting clinically as renal cell carcinoma – A case report

We describe a 22-year old female who presented with a 5-year history of a palpable, painless mass in the right flank. Computerized tomography demonstrated a solid renal mass measuring 18 cm × 13 cm with peripheral calcification, areas of vascularity and necrosis. The appearance suggested renal cell carcinoma or nephroblastoma, but percutaneous renal biopsy suggested an adrenal origin. At right radical nephrectomy, the adrenal gland was completely normal. Histology showed sheets and nests of epithelioid cells with abundant eosinophilic to clear cytoplasm, confirming a diagnosis of epithelioid angiomyolipoma (EAML), a rare mesenchymal tumor belonging to the perivascular epithelioid cell tumor family (PEComas). At 33 months followup, there was no evidence of recurrence or metastases.