The uniqueness of ccRCC metabolic networks has to be ascribed to the loss of chromosome 3p encompassing all of the four commonly mutated genes (i.e., VHL, PBRMI, BAP1 and SETD2) leading to alterations in genes coding for chromatin remodeling proteins and in genes controlling cellular oxygen sensing [89]. Here, VHL is linked to nonpapillary renal cell carcinoma.