In familial studies of patients with schwannomatosis and multiple meningiomas, germline mutations in the tumor suppressor gene SMARCB1 (SWIch/Sucrose Non-Fermentable (SWI/SNF)-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1) and NF2 were identified as the key predisposing alterations [18–21]. This evidence concerns the gene NF2 and meningioma.