SETD2 and von Hippel-Lindau disease: SPORADIC:3p loss/deletions or biallelic alteration of VHL gene (3p25) by mutation or hypermethylation (80–98%).ccRCC tumor suppressor genes harbored by 3p locus: KD-M6A (or UTX), KDM5C (or JARID1C), SETD2 and PBRM1.Loss of chromosome 4p, 8p, 9p, 14q. Gain of chromosome 5q.Mutations in BAP1 and PBRM1 genes.FAMILIAL:Von Hippel-Lindau disease;constitutional chromosome 3 translocations.