Not only somatic but also germline mutation of BAP1 gene may occur in UM and the majority of familial UM with germline BAP1 mutation is associated with BAP1-tumor predisposition syndrome, which also increases the risk of the atypical Spitz tumors, malignant mesothelioma, and cutaneous melanoma43. Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.