Long-term followup after nephron sparing surgery for renal cell carcinoma in von Hippel-Lindau disease.

We reviewed the long-term outcome of nephron sparing surgery in 9 patients with localized bilateral renal cell carcinoma and von Hippel-Lindau disease. One patient died of metastatic renal cell carcinoma 43 months postoperatively. One patient has not had recurrent tumor and was alive at 74 months postoperatively. The remaining 7 patients (mean followup 88 months) had local recurrence of tumor in the operated kidney and a secondary renal operation was done in 6 of them. Overall, 6 patients are free of tumor but only 3 of them retain functioning native renal parenchyma. We conclude that the results of nephron sparing surgery in patients with renal cell carcinoma and von Hippel-Lindau disease are less satisfactory than in patients with sporadic renal cell carcinoma.