Progress in Nephron Sparing Therapy for Renal Cell Carcinoma and von Hippel-Lindau Disease

PurposePatients with von Hippel-Lindau disease frequently have early, multiple and recurrent renal cell carcinoma. Renal cell carcinoma treatment, which must prevent metastatic disease and spare nephrons, has changed in the last 2 decades. We evaluated renal cell carcinoma treatments in the long term in a large series of patients with von Hippel-Lindau disease.Materials and MethodsWe retrospectively evaluated the use and results of surgery and radio frequency ablation in patients with von Hippel-Lindau followed at our institution between 1988 and 2009. Renal anatomical survival was analyzed according to 3 periods, including 1) 1988 to 1994—the learning phase of nephron sparing surgery, 2) 1995 to 2003—routine nephron sparing surgery and 3) 2004 to 2009—the emergence of radio frequency ablation.ResultsA first renal cell carcinoma was treated at a mean age of 38 years (range 15 to 67) in 113 patients with von Hippel-Lindau disease. During a median followup of 7.2 years 251 therapeutic procedures were performed in a total of 176 kidneys. We observed a shift of first line renal cell carcinoma treatment with time, that is nephrectomy in 52% of cases in period 1, tumorectomy in 75% in period 2 and radio frequency ablation in 43% in period 3. The shift paralleled improved renal survival. While nephron sparing surgery was primarily done for lesions greater than 30 mm, radio frequency ablation was used to treat less numerous and smaller ipsilateral lesions but they required more frequent intervention. Radio frequency ablation became the most widely used second or third line procedure and allowed renal salvage in 8 patients.ConclusionsNephron sparing surgery and more recently radio frequency ablation enable earlier treatment of smaller tumors and are associated with a significant improved renal prognosis in patients with von Hippel-Lindau disease.