Surgical Management of von Hippel-Lindau Disease: Urologic Considerations

Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited tumor syndrome in which a genetic defect in the VHL gene is located on chromosome 3p25. The urologic surgeon is an integral part of the management team for patients who have VHL disease, because patients frequently have multiple urologic tumors. This article presents a cumulative review of the literature regarding the diagnosis and management of urologic tumors in patients who have VHL disease, along with the latest data regarding the genetics and molecular mechanisms of VHL disease.