Purpose: We reviewed the efficacy and safety of nephron sparing surgery for renal cell carcinoma in patients with von Hippel-Lindau disease.
Materials and methods: Data were collated for all 56 patients with a mean age of 37.2 +/- 11.3 years (range 14 to 62) with von Hippel-Lindau disease who underwent radical nephrectomy or nephron sparing surgery at our department for 1 or more 25 to 60 mm renal cell carcinomas between 1988 and 2001.
Results: Overall 30 nephrectomies (33%) and 62 enucleations (67%) were performed for 62 bilateral and 30 unilateral tumors. For nephron sparing surgery estimated intraoperative blood loss was 175 +/- 231.7 cc (range 50 to 1,300), 97% of surgeries had vascular pedicle clamping for 32 +/- 10.4 minutes (range 10 to 50) and there were 4 immediate complications (1 perinephric abscess, 2 urinary fistulas and 1 acute renal failure requiring temporary dialysis). Renal atrophy was noted in 7.3% of cases. Tumor diameter was 31.2 +/- 10.7 mm (range 15 to 60) and recurrence diameter was 22 +/- 7.8 mm (range 4 to 45). Hospital stay was 7.6 +/- 2.4 days (range 5 to 21). Preoperative and postoperative creatinine was 1.0 +/- 0.2 (range 0.6 to 1.7) and 1.2 +/- 0.9 mg/dl (range 0.7 to 6.5), respectively. Median followup was 55.9 months. There were 17 local recurrences (27.4%) and no metastases at recurrence. The overall survival rate was 100% at 5 years and 67% at 10 years.
Conclusions: Nephron sparing surgery is effective and, when feasible, it need not be called into question. However, it may probably be superseded by less invasive techniques for tumors less than 20 mm diagnosed early after these techniques have been validated in long-term trials.