Germline pathogenic mutations in the tumor suppressor gene BRCA1-associated protein-1 (BAP1) lead to BAP1 tumor predisposition syndrome (BAP1-TPDS) [6], which is characterized by high susceptibility to several tumor types, mainly melanoma (especially uveal), mesothelioma, renal cell carcinoma, and basal cell carcinoma (Table 2). This evidence concerns the gene BAP1 and hereditary clear cell renal cell carcinoma.