Renal Lesions in von Hippel-Lindau Disease: The Benign, the Malignant, the Unknown

Abstract

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.