Somatic mutation in this gene is observed in many cancers including breast, lung and mesothelioma and germline mutations have been found in familial uveal melanoma and mesothelioma cases.86,87 It has been shown that BAP1 mutation appears in the late stages of tumorigenesis, causes changes in phenotype, and is associated with metastatic behavior and class 2 genetic structure in 84% of patients.88,89 Mutation of one allele is usually accompanied by loss of the other entire copy of chromosome 3. Here, BAP1 is linked to mesothelioma.