BAP1-TPDS should be suspected if an individual has been diagnosed with two or more tumors of the BAP1-TPDS spectrum or has one BAP1-TPDS malignancy and a first- or second-degree relative with a tumor included in the BAP1-TPDS spectrum, has a personal history of two or more inactivated melanocytic tumors (BIM), or developed mesothelioma at a young age (less than 60 years) [34,35]. Here, BAP1 is linked to mesothelioma.