Background: Renal cell carcinoma is a frequent cause of morbidity and mortality in patients with von Hippel-Lindau disease.
Methods: A review was conducted of 10 patients with von Hippel-Lindau disease and localized renal tumors, who underwent renal parenchyma-sparing surgery. Four patients had recurrences, requiring a total of 16 operations. Patients 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 preoperative imaging studies. This was accomplished primarily by enucleation or partial nephrectomy when anatomically feasible. No patient required 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 cancer with recurrent renal cell carcinoma found at autopsy, and one of metastatic renal cell carcinoma. Another patient with probable recurrent tumor was being observed at the time of this writing. The other seven patients were alive and without evidence of renal cancer.
Conclusion: Although the risk of recurrence is high, these patients may be treated with parenchyma-sparing renal surgery and enjoy a prolonged survival, and avoid the complications of dialysis or transplantation.