The most common benign brain Tumours, meningioma, accounts for about 15 % of all primary brain Tumours. The frequency of meningioma increases with advancing age, and it is more common in women. Multiple or familial meningioma is associated with (a) neurofibromatosis type 2 (NF2), (b) pure familial meningioma, (c) constitutional chromosome 22 rearrangements, and
(d) familial schwannomatosis and SMARCB1 mutations. Meningioma also occurs with increased frequency in Werner syndrome and Gorlin syndrome.
Multiple meningioma is frequent and occurs in about a third of patients with NF2. Expression of NF2 is variable, so a careful search for evidence of NF2 and a detailed family history should be performed in all patients with multiple or familial meningioma, or with a young age at onset. Although many reports of familial meningioma may be variants of NF2, dominantly inherited meningioma with no evidence of NF2 does occur.
However, signs of NF2 should be assiduously sought in all cases of familial meningioma as these may not be obvious. For example, Delleman et al. (1978) reported a family in which four members in two generations had meningiomas with no evidence of neurofibromatosis, but another relative had multiple meningiomas and bilateral vestibular schwannomas.
Rearrangements of chromosome 22 have been associated with meningioma: multiple Tumours developed in the third decade in a mentally retarded patient with a ring chromosome 22 (breakpoints p12 and q13.3) (Arinami et al. 1986), and familial meningiomas associated with a Robertsonian chromosome 14;22 translocation have also been described. In addition, Pulst et al. (1993) reported exclusion of linkage to the NF2 kindred with familial meningioma.
Germline SMARCB1 mutations have been identified in patients with a combination of multiple meningiomas and schwannomatosis (van den Munckhof et al. 2012). However, among a cohort of patients with multiple meningiomas and no schwannomas, germline SMARCB1 mutations appeared to be rare (Hadfield et al. 2010) though Smith et al. (2013) described SMARCE1 mutations in kindreds with familial spinal meningiomas with clear cell histology.