Malignant renal tumors in tuberous sclerosis

Although the frequency of renal carcinoma in tuberous sclerosis is lower than that in von Hippel-Lindau syndrome, it occurs bilaterally in 43 percent of patients at a median age of twenty-eight years. Concomitant hamartomas and hyperplastic cystic components may confound the histologic diagnosis of renal carcinoma in tuberous sclerosis.