Renal cell carcinoma in von Hippel-Lindau syndrome

Thirty-seven cases of von Hippel-Lindau syndrome are reported. The urinary tract was studied in 23 patients; 15 of them had the renal lesions. Eight of these 15 patients had renal cell carcinoma, which was bilateral in six. Because 80 percent of these patients are seen initially by neurologists or ophthalmologists, the potential for the development of renal cell carcinoma in these patients, the familial nature of the disorder, and the need for early urologic investigations and subsequent close follow-up are emphasized.