VHL and nonpapillary renal cell carcinoma: Clear cell renal cell carcinoma (ccRCC) comprises about 3% of adult cancer cases and is an aggressive tumor characterized in most cases by the inactivation of the tumor -suppressor gene VHL. The Von Hippel–Lindau protein (pVHL) regulates the hypoxia-inducible factor (HIF), and the loss of the pVHL function causes constitutive stabilization of HIF-1α and HIF-2α, resulting in the induction of HIF-transcriptional targets [2].