EPITHELOID TYPE RENAL ANGIOMYOLIPOMA

Introduction: Angiomyolipoma (AML) is a well-known rare soft tissue tumor involving the kidneys, liver and other organs. For a long time believed to be a benign hamartoma, angiomyolipoma is now considered a neoplasm that arises from perivascular epitheloid cell. Epitheloid angiomyolipoma is a rare variant of angiomyolipoma characterized by epitheloid cells that mimic renal cell carcinoma and it is potentially malignant. Immunreactivity with HMB45 and CD68 are helpful for its identification. We report a multifocal epitheloid angiomyolipoma case with prominent pleomorphism and necrosis and reviewed literature.