The von Hippel-Lindau gene is the archetype of the tumor suppressor gene and when inactivated cell growth is unchecked and tumors result.
Renal cysts are very common.
They are often multiple and bilateral and vary in size from several millimeters to more than 2 cm. The latter tumor type are a major cause of morbidity and mortality in vHL patients, and follow cerebellar and retinal lesions in frequency.