Pure epithelioid angiomyolipoma of kidney in tuberous sclerosis patient: A case report and review of literature

Background:The majority of renal angiomyolipoma (AML) is sporadic and occasionally it occurs in background of tuberous sclerosis complex (TSC). Epithelioid AML (EAML) is an uncommon variant, with uncertain behavior. Case presentation:We present a case of tuberous sclerosis with right large renal tumor. The tumor revealed epithelioid cells accompanied by thick wall vessels and muscular bundles in all the sections; devoid of lipocytes. Immunohistochemistery confirmed the diagnosis of epithelioid PEComa. Conclusion:Pure EAML is a rare tumor with potential malignancy. Accurate diagnosis especially differentiation from renal cell carcinoma, is very important for proper management of patient