To evaluate the risks of local recurrence and repeat surgery after nephron-sparing surgery (NSS) in von Hippel-Lindau (VHL) disease to propose a therapeutic strategy to patients.

A total of 21 patients with VHL disease and renal tumors were followed up from February 1987 to August 2005. Surgical resection of all tumors was chosen when the largest tumor exceeded a diameter of 30 mm.

Of the 21 patients, 18 underwent surgery at a mean age of 38.5 years (range 24 to 69). The median follow-up of the series was 100 months. Of the 17 patients treated by NSS, 8 developed a recurrence. The mean time to local recurrence was 53 ± 38.8 months (range 10 to 115), and the recurrence tumors grew at a stable mean rate of 0.34 ± 0.32 cm/yr (range 0.1 to 1.08). Repeat NSS was performed on the same kidney in 2 cases and eight recurrences were kept under surveillance. The disease-specific survival rate was 93.8% at 10 years. The local recurrence rate was 45.6% at 5 years and 83.7% at 10 years. The overall repeat surgery rate was 23.1% at 5 years and 63.4% at 10 years. No metastasis or chronic renal insufficiency was observed in patients undergoing NSS only.

Five years after NSS, the risk of developing new tumors in the same kidney and the overall risk of repeat surgery was about 50% and 25%, respectively. However, the strategy of elective NSS and close surveillance preserved renal function without increasing the risk of metastasis.