Extrarenal angiomyolipoma with melanocytic and hibernoma-like features

Angiomyolipoma is a tumor commonly occurring in the kidney, but occasionally found in extrarenal sites. Retroperitoneal angiomyolipoma with unusual features presenting in a 39 year old woman with hypertension is reported in this paper. Tumor fat was inconspicuous, and present largely as hibernoma-like microvesicular lipid. Tumor cells also demonstrated positivity for HMB-45 and S-100 protein, and by electron microscopy showed occasional cytoplasmic striated granules indistinguishable from stage II premelanosomes. However, electron microscopy and immunocytochemistry also confirmed the presence of a substantial myogenous component in the tumor, establishing the diagnosis of angiomyolipoma. The implications of these findings, and the role of immunocytochemistry and electron microscopy in the diagnosis of this tumor are discussed.