BAP1 and Miyoshi myopathy: Mutations in CDKN2A/B were found to be the most associated co-alteration in aggressive NF2mut meningiomas, seen in 24% of cases.37SMARCB1 mutations were also found in NF2mut intraventricular meningioma.39 Recently, a mouse model of MM was generated based upon disruption of the NF2, BAP1, and CDKN2A/B tumor suppressor loci in various combinations as also frequently observed in human MM.40 Inactivation of all 3 loci in the mesothelial lining of the thoracic cavity led to a highly aggressive MM that recapitulates the histologic features and gene expression profile observed in human MM.