PURPOSE: Patients 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 METHODS: We 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.
RESULTS: A 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.
CONCLUSIONS: Nephron 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.