Angiomyolipoma with epithelial cysts of the kidney in a man

Angiomyolipoma (AML) is a well-characterized tumor composed of vascular, smooth muscle and adipose elements. Recently, AML with epithelial cysts (AMLEC) has been described as a cystic variant of AML. Herein is reported an AMLEC in a 55-year-old man, and to the authors' knowledge this is the first case report of AMLEC in Japan. He was referred to hospital because of microhematuria. A left renal tumor was noted and partial nephrectomy was performed. Histology indicated that the tumor was composed of epithelial cysts, compact subepithelial stroma and muscle-predominant AML. Immunohistochemically, the subepithelial compact stroma and muscle-predominant AML were positive for HMB-45, estrogen receptor (ER), progesterone receptor (PR), CD10, smooth-muscle actin and desmin. Epithelial cysts were positive for pancytokeratin but negative for HMB-45, ER and PR. This tumor was diagnosed as AMLEC, mainly based on the immunohistochemical result that subepithelial compact stroma and muscle-predominant AML were positive for HMB-45. The patient was alive with no evidence of recurrence at 2 years postoperatively. This case was very interesting because it suggests that AMLEC should enter into the differential diagnosis of adult renal neoplasms.