The von Hippel–Lindau (VHL) mutation shows that it is able to increase susceptibility to hemangioblastoma in the retina or central nervous system, neuroendocrine tumors (notably pheochromocytoma), and familial or sporadic clear-cell RCC (ccRCC) at a high frequency in humans [46]. The gene discussed is VHL; the disease is nonpapillary renal cell carcinoma.