CONSERVATIVE SURGICAL THERAPY OF LOCALIZED RENAL-CELL CARCINOMA IN VON HIPPEL-LINDAU DISEASE

Background. Renal cell carcinoma is a frequent cause of morbidity and mortality in patients with von Hippel-Lindau disease. Methods. A revie w was conducted of 10 patients with von Hippel-Lindau disease and loca lized renal tumors, who underwent renal parenchyma-sparing surgery. Fo ur patients had recurrences, requiring a total of 16 operations. Patie nts were followed for recurrence by computed tomography. Results. The mean follow-up for these patients was 62 months (range, 11-118 months) . Seventy-two tumors were resected, including 36 tumors not seen by pr eoperative imaging studies. This was accomplished primarily by enuclea tion or partial nephrectomy when anatomically feasible. No patient req uired dialysis, and the mean serum creatinine at follow-up was 1.2 mg/ dl (range, 0.9-1.7). Of the 10 patients, 2 died, one of primary lung c ancer with recurrent renal cell carcinoma found at autopsy, and one of metastatic renal cell carcinoma. Another patient with probable recurr ent tumor was being observed at the time of this writing. The other se ven patients were alive and without evidence of renal cancer. Conclusi on. Although the risk of recurrence is high, these patients may be tre ated with parenchyma-sparing renal surgery and enjoy a prolonged survi val, and avoid the complications of dialysis or transplantation.