In good agreement with the higher prevalence of BAP1 loss in epithelioid mesothelioma, germline BAP1 mutations, characterizing BAP1 tumor predisposition syndrome, typically give rise to epithelioid mesotheliomas, which paradoxically appear to have a less aggressive behavior and a prolonged median survival (~5–7 years) [45,46]. Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.