Numerous studies have now confirmed and expanded on the direct link of BAP1 germline mutations to a hereditary cancer syndrome characterized by a predisposition to mesothelioma, uveal melanoma, and less frequently cutaneous melanoma, as well as clear cell renal cell carcinoma 21, which are the core cancer types in the BAP1 cancer syndrome. This evidence concerns the gene BAP1 and clear cell renal carcinoma.