Urological management of patients with von Hippel-Lindau's disease

We present our experience with 7 patients with von Hippel-Lindau's disease. Five patients had bilateral renal tumors and 2 had pheochromocytomas. Staging is accomplished best with abdominal computerized tomography and renal angiography. Computerized tomography is the preferred method to follow the patients. Because of the tendency for these patients to have bilateral renal involvement, surgical efforts should be directed toward preserving renal parenchyma without compromising adequate tumor excision. Of the 7 patients 6 are alive 4 months to 8 years postoperatively. One patient had metastatic disease at autopsy. No patient required dialysis or renal transplantation. The relationship between the multifocal renal cysts and renal carcinomas in this disease remains unknown.