Cranial meningiomas in 411 neurofibromatosis type 2 (NF2) patients with proven gene mutations: clear positional effect of mutations, but absence of female severity effect on age at onset

Neurofibromatosis type 2 (NF2) is caused by mutations in the NF2 gene. Around half of NF2 patients in various studies have been shown to have meningiomas, although the factors determining which individuals will develop meningiomas are unclear. We analysed the correlation between incidence of cranial meningiomas and mutation position within the NF2 gene in 411 NF2 patients.

We found a clear effect of mutation position with risk of meningioma and no overall difference in risk between men and women, although men had a slightly increased risk below age 20. This information is of value when counselling patients about their risk of cranial meningioma. (By Dr. Miriam J Smith, http://jmg.bmj.com/content/early/2011/01/28/jmg.2010.085241?papetoc=)