Heterozygous germline mutations of BAP1 confer increased susceptibility for the development of several tumors, mostly uveal and cutaneous melanomas, epithelioid atypical Spitz tumors, and mesotheliomas but also other neoplasms, including renal cell carcinoma, lung adenocarcinoma, and meningioma (BAP1-TPDS, OMIM 614327) [103]. Here, BAP1 is linked to hereditary clear cell renal cell carcinoma.