Objectives: To describe the features and treatment of renal lesions in
von Hippel-Lindau disease (VHL) from a series of patients, to highlig
ht important issues in the management of these lesions. Materials and
Methods: We performed a retrospective study in 7 patients with VHL who
underwent surgery for renal lesions between January 1990 and July 199
6. The initial evaluation consisted of an abdominal CT scan and renal
arteriography. Results: The mean age of patients at the time of discov
ery was 38 (+/- 12.01 years). AU cases were of type 1. Radiology asses
sment underestimated the gravity of the lesions in 5 patients. In all
patients, unilateral surgery was performed with the kidney being conse
rved in 4 cases. Of the 44 lesions removed, 23 had signs of progressiv
eness (atypical or malignant cyst, renal cell carcinoma), most of the
cancers were of low grade. 84% of the lesions did not exceed 3 cm and
48% were less than or equal to 1 cm. During follow-up (42 months), 2 p
atients relapsed. Conclusion: The clinical and pathological features o
f renal lesions in VHL require strict and early follow-up (from the ag
e of 15). Only under these circumstances should primary conservative s
urgery be performed, however, recurrences are frequent. This conservat
ive approach remains to be validated by multicentric prospective studi
es.