Objectives: We report our experience of renal and/or adrenal manifestations of von Hippel-Lindau disease and propose a practical approach.
Methods: Eight patients (mean age: 43 years) presented with predominant renal and adrenal lesions in 6 cases and 2 cases, respectively.
Results: All patients are alive with a mean follow-up of 8.1 years. A local recurrence after partial nephrectomy was observed in two cases. Two patients are in renal failure and are treated by dialysis and two patients require hormone replacement therapy for adrenal insufficiency.
Conclusions: The predegenerative nature of simple renal was not observed. Conservative renal surgery is adapted to small renal tumours, with a low cytological grade and without any distant lesions in the same kidney. Radical nephrectomy is reserved for large lesions (greater than 5 cm) with a high cytological grade. The presence of pheochromocytoma must be systematically excluded. Preservation of the adrenal gland in the case of homolateral renal surgery for cancer is recommended. The reliability of the genetic test allows early diagnosis of this disease.
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