Epithelioid Angiomyolipoma of kidney - An uncommon tumor mimicking renal cell carcinoma .

Abstract

Introduction: Epithelioid angiomyolipoma (EAML) is an uncommon variant of angiomyolipoma (AML) with malignant potential, frequently associated with tuberous sclerosis complex. Only few cases have been reported so far in the literature. Preoperative diagnosis is difficult. Differential diagnosis from renal cell carcinoma is often challenging because of its epithelioid morphology and carcinoma like growth pattern. Case report: A 52 year old man presented with progressive weight loss and abdominal pain .CT scan showed a large renal mass on right side .With a radiological diagnosis of renal cell carcinoma he underwent laparoscopic nephrectomy. With histopathological and immunohistochemical features a diagnosis of epithelioid angiomyolipoma was made .Retrospectively we came to know that he is a known case of tuberous sclerosis Conclusion: EAML should be a differential diagnosis when we are dealing with renal tumors in young patients with tuberous sclerosis and with epithelioid morphology and high nuclear grade. It is important to have a high index of suspicion so that a correct diagnosis can potentially direct clinician to a more effective targeted treatment using mTOR inhibitors especially in TSC associated cases.