VHL and neoplasm: Germline inheritance of a single mutant allele of VHL gives rise to the dominantly inherited VHL familial cancer syndrome which predisposes not only to the formation of ccRCC, but also to cystic lesions in the kidney and pancreas as well as to diverse types of tumours in the central nervous system, eye, ear, pancreas, adrenal gland, epididymis and broad ligament (Kaelin, 2002).