Renal cell carcinoma within an angiomyolipoma was diagnosed in a 21-year-old man with tuberous sclerosis. Ultrasound characteristics are discussed. A review of the 21 cases reported since 1969 suggests an increased incidence of renal cell carcinoma in patients with tuberous sclerosis. Genetic studies in humans and animals support this observation. Awareness of predisposition to renal carcinoma is important to help identify this malignancy in patients with tuberous sclerosis and to differentiate carcinoma from other renal lesions that commonly occur in these patients.
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