Renal cell carcinoma associated with renal angiomyolipoma and renal cortical adenoma

OBJECTIVE: To report a case of renal cell carcinoma associated with renal angiomyolipoma and renal cortical adenoma and discuss the possibility of a hereditary etiology. METHODS: A 64-year-old patient with a tumor in the right kidney underwent right renal nephrectomy. The pathological findings revealed a clear cell renal carcinoma, two cortical angiomyolipoma and a renal cortical adenoma. The literature on these three coincident renal tumors is reviewed. RESULTS: The association of these three renal tumors is rare and is usually seen in patients with tuberous sclerosis. CONCLUSIONS: Renal angiomyolipoma is a benign tumor frequently associated with Bourneville's disease or tuberous sclerosis, a disorder of autosomal dominant transmission. Familial evidence of renal cell carcinoma has been previously reported, as in von Hippel-Lindau disease. It has been suggested that heredity may play an important role in the association of renal cell carcinoma and other renal tumors.