[Renal cell carcinoma in von Hippel-Lindau disease. Nephron sparing surgery.]

We reviewed the feasibility and safety of nephron sparing surgery for renal cell carcinoma in patients with von Hippel Lindau (VHL) disease.

We selected 22 patients with (VHL) disease with a mean age of 43 (range 30-56), from whom 16 underwent radical nephrectomy or nephron sparing surgery at our department between 2000 and 2015.

A total of 33 tumors were treated, by either tumorectomy (n=20), partial nephrectomy (n=5), percutaneous renal radiofrequency ablation (n=3) edaor radical nephrectomy (n=5). All procedures were successfully completed without intraoperative and postoperative complications. The diameter of the tumor ranged from 2.8 to 4.8 cm. The interval between treatments in patients operated more than once was 40 months. Renal function stayed stable with basal creatinine and current creatinine 0.74 ± 0.21 mg/dl and 0.93 ± 0.22 mg/dl respectively. Median follow-up was 72.3 months. Cancer-specific survival was 97%.

nephron sparing surgery in renal tumors >3 cm is an effective and safe treatment for VHL patients.