VHL and renal cell carcinoma: Clear cell renal cell carcinoma (ccRCC), derived from the epithelial cells in the nephron, is the predominant subtype of renal cell carcinoma (RCC) (up to 80% of all RCCs), and characterized by the inactivation of the von Hippel Lindau (VHL) gene and subsequent dysregulation of hypoxia-inducible factor (HIF)-responsive genes (1–4).