VHL and nonpapillary renal cell carcinoma: For example, mutations in the VHL tumor suppressor cause the autosomal dominant familial cancer syndrome von Hippel-Lindau disease (VHL) that is characterized by the development of renal and pancreatic cysts, ccRCC, pheochromocytoma (tumor of the adrenal gland), cysts and hemangiomas in the central nervous system, retinal angiomas, and low-grade adenocarcinomas of the temporal bone [178].