Treatment of Renal Cell Carcinoma in Von Hippel-Lindau Disease: A Multicenter Study

AbstractA total of 65 patients with von Hippel-Lindau disease underwent surgery for renal cell carcinoma (54 bilaterally and 11 unilaterally) at 8 medical centers. Only 1 patient presented with metastatic disease. Radical nephrectomy and nephron sparing surgery were performed in 16 and 49 patients, respectively. Mean posttreatment followup was 68 months.The 5 and 10-year cancer-specific survival rates for all patients were 95 percent and 77 percent, respectively. The corresponding rates for patients treated with nephron sparing surgery were 100 percent and 81 percent, respectively. Of the latter patients 25 (51 percent) had postoperative local tumor recurrence but only 2 had concomitant metastatic disease. Survival free of local recurrence was 71 percent at 5 years but only 15 percent at 10 years. End stage renal failure occurred in 15 patients (23 percent): 6 underwent renal transplantation (5 are alive with satisfactory renal function and no evidence of malignancy) and 9 were treated with chronic dialys...