Patients with a germline mutation in the VHL gene develop clear cell renal cell carcinoma (ccRCC), retinal and central nervous system hemangioblastomas, pheochromocytomas, pancreatic neuroendocrine tumors, cystadenomas of the pancreas and middle ear, and erythrocytosis (Maher and Kaelin, 1997). This evidence concerns the gene VHL and nonpapillary renal cell carcinoma.