Mutations in the VHL gene are known to be important in renal cancers; this includes the occurrence of clear cell renal cell carcinoma (ccRCC) as part of the inherited cancer syndrome von Hippel–Lindau disease, in which VHL is mutated and PCC/PGL can occur (Gossage et al. 2015, Crespigio et al. 2017). This evidence concerns the gene VHL and von Hippel-Lindau disease.