Malignant renal epithelioid angiomyolipoma (EAML): about a rare case

Malignant renal epithelioid angiomyolipoma (EAML) is a rare disease which has long been considered a hamartomatous lesion. Positive diagnosis is purely based on immunohistochemical investigation. Microscopically, it is characterized by mononuclear epithelioid cells showing a clear cytoplasm with severe cytonuclear atypies and expressing smooth muscle cell markers and melanocytic markers (HMB 45). We here report a rare case of EAML diagnosed after anatomopathological examination of a retroperitoneal tumor whose origin wasn't predetermined by CT scan.