Subsequent research demonstrated germline BAP1 mutations with familial clustering in different neoplasias, leading to the discovery of a new cancer syndrome, termed BAP1-tumour predisposition syndrome, which leads to the development of benign and malignant melanocytic skin tumours, malignant mesothelioma, UM and renal cell carcinomas, among other neoplasias [41,49,50,51,52]. Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.