In this context, we read with great interest the article of Vimercati and colleagues (1), who described a case of pleural mesothelioma (PM) in an 83-year-old man, with a story of environmental and working story of asbestos exposure, suspect for BAP1 tumor predisposition syndrome (BAP1-TPDS). This evidence concerns the gene BAP1 and BAP1-related tumor predisposition syndrome.