Abstract
Renal angiomyolipoma (AML) is recognized as a benign hamartomatous lesion arising in the kidney with no obvious malignant potential. However, epithelioid AML (EAML), a rare variant of AML, is potentially malignant, with aggressive clinical features. It can occur in patients with or without tuberous sclerosis. Because EAML may mimic renal cell carcinoma in imaging studies, differentiation of this tumor from renal cell carcinoma preoperatively is difficult. At times, the lesions may extend into the renal vein and inferior vena cava or metastasize to other organs such as the lung and liver. To clarify the biological nature of EAML, three specific cases that we encountered in clinical practice are analyzed and reported in detail.