Epithelioid renal angiomyolipoma

To issue the existence of a variety of angiomyolipoma, named epithelioid, with different histological and clinic properties. We report the case of a 17 year old female, with Bourneville's disease, who was discovered to have a solid renal mass suggestive of carcinoma in a control CT scan, adjacent to other smaller masses identified as angiomyolipomas. After several tumorectomies, the suspicious mass, 4 cm in size, was diagnosed as epithelioid angiomyolipoma, with immunohistochemical confirmation of capacity for HMB45, and negative vimentin and keratin. Despite the possibility of coexistence of adenocarcinoma and angiomyolipoma, the existence of an epithelioid variety cannot be discarded, mainly in patients with phakomatosis. The indications for surgery are the same than for the rest of renal masses. Nevertheless, follow-up criteria must be stricter due to the possibility of torpid outcome in terms of dissemination of this infrequent variety of angiomyolipoma.