Renal lesions in Von Hippel-Lindau disease: the benign, the malignant, the unknown.

OBJECTIVES

To describe the features and treatment of renal lesions in von Hippel-Lindau disease (VHL) from a series of patients, to highlight 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 1996. The initial evaluation consisted of an abdominal CT scan and renal arteriography.

RESULTS

The mean age of patients at the time of discovery was 38 (+/- 12.01 years). All cases were of type 1. Radiology assessment underestimated the gravity of the lesions in 5 patients. In all patients, unilateral surgery was performed with the kidney being conserved in 4 cases. Of the 44 lesions removed, 23 had signs of progressiveness (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 </=1 cm. During follow-up (42 months), 2 patients relapsed.

CONCLUSION

The clinical and pathological features of renal lesions in VHL require strict and early follow-up (from the age of 15). Only under these circumstances should primary conservative surgery be performed, however, recurrences are frequent. This conservative approach remains to be validated by multicentric prospective studies.