Nine cases of UM were found in 72 BAP1-TPDS patients (13%), 13 carriers with BAP1-TPDS were diagnosed with CM (18%), 8 with MMe (11%), and 2 with RCC (3%), while the recent worldwide meta-analysis found an occurrence of 25%, 20%, 20%, and 6% for UM, CM, MMe, and RCC, respectively [12]. Here, BAP1 is linked to cutaneous mastocytosis.