VHL and clear cell renal carcinoma: Inactivation of the von-Hippel Lindau (VHL) tumor suppressor gene is the initiating or “truncal” event in nearly 90% of sporadic clear cell renal cell carcinomas and in all ccRCCs that develop in the setting of the hereditary cancer syndrome, von Hippel–Lindau (VHL) disease (2, , , –6).