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Significance Survival from malignant mesothelioma, particularly pleural mesothelioma, is very poor. For patients with some cancers, overall survival following platinum chemotherapy is better for patients with germline mutations in DNA repair and related genes than for patients without such mutations. We evaluated this relationship for patients with malignant mesothelioma. Following platinum chemotherapy, overall survival was significantly longer for patients with loss-of-function mutations inBAP1 and DNA repair genes compared with patients with no such mutations. The effect of genotype was highly significant for patients with pleural mesothelioma, but not for patients with peritoneal mesothelioma, and remained significant after adjusting for gender and age at diagnosis. These patients may benefit from DNA repair targeted therapies such as poly-ADP ribose polymerase inhibitors.

Inherited predisposition to malignant mesothelioma and overall survival following platinum chemotherapy