VHL is a key tumor suppressor that is mutated in Von Hippel-Landau disease, a hereditary cancer predisposition syndrome that often manifests as clear-cell renal carcinoma (ccRCC) (Chen et al., 1995; Gossage et al., 2015; Kim and Kaelin, 2004; Maher et al., 1990). This evidence concerns the gene VHL and nonpapillary renal cell carcinoma.