Meningiomas arise from the arachnoid layer of the meninges that surround the 
brain and spine. They account for over one third of all primary central nervous 
system tumors in adults and confer a significant risk of location-dependent 
morbidity due to compression or displacement. A significant increase in risk of 
meningiomas is associated with neurofibromatosis type 2 (NF2) disease through 
mutation of the NF2 gene. In addition, approximately 5% of individuals with 
schwannomatosis disease develop meningiomas, through mutation of the SWI/SNF 
chromatin remodeling complex subunit, SMARCB1. Recently, a second SWI/SNF 
complex subunit, SMARCE1, was identified as a cause of clear cell meningiomas, 
indicating a wider role for this complex in meningioma disease. The sonic 
hedgehog (SHH)-GLI1 signaling pathway gene, SUFU, has also been identified as 
the cause of hereditary multiple meningiomas in a large Finnish family. The 
recent identification of somatic mutations in components of the SHH-GLI1 and 
AKT1-MTOR signaling pathways indicates the potential for cross talk of these 
pathways in the development of meningiomas. This review describes the known 
meningioma predisposition genes and their links to the recently identified 
somatic mutations.