Nephron-sparing surgery and renal transplantation in patients with renal cell carcinoma and von Hippel-Lindau disease.

Renal cell carcinoma is a common manifestation of von Hippel-Lindau disease and an important cause of mortality in these patients. A contemporary approach to the treatment of localized renal cell carcinoma is outlined based upon several recently conducted multicentre reviews. When technically feasible nephron-sparing surgery can preserve renal function without compromising long-term survival in most patients. For those who develop end-stage renal failure as a result of treatment for renal cell carcinoma, excellent results with a limited risk for development of recurrence can be obtained with renal transplantation.