NF2 and cancer: This hypothesis is in line with several findings: (i) inactivation of NF2 in mouse models led to a variety of malignant tumors but not to mesothelioma [51], except if this inactivation was associated with asbestos exposure or with the inactivation of other tumor suppressor genes [52]; recent studies screening germline mutations in large cohorts of patients (reviewed in [44]) did not identify NF2 as a cancer susceptibility gene for MPM; and (iii) NF2 mutations showed a significantly higher mutation rate in MPM with an advanced stage [22].