Multiple meningiomas: differential involvement of the NF2 gene in children and adults

J Med Genet. 2005 Jan;42(1):45-8. doi: 10.1136/jmg.2004.023705.

Abstract

Objective: To screen for NF2 mutations in people with meningiomas.

Methods: Lymphocyte or tumour DNA was analysed from 46 individuals from 36 families who presented with a meningioma at age < or =15 years without vestibular schwannoma (VS), or who had multiple meningiomas in adulthood before the diagnosis of VS.

Results: Eight of 13 people with meningioma and other features of neurofibromatosis 2 (NF2) had an identified constitutional NF2 mutation in blood DNA, but none of the other subjects had identified constitutional NF2 mutations.

Conclusions: Constitutional NF2 mutations are the most likely cause of meningioma in children and in people with a meningioma plus other non-VS features of NF2. Mosaic NF2 may be the cause of about 8% of multiple meningiomas in sporadic adult cases, but there are other causes in the majority of other such patients and in multiple meningioma in families.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Genes, Neurofibromatosis 2*
  • Humans
  • Loss of Heterozygosity
  • Meningioma / genetics*
  • Mosaicism
  • Mutation*
  • Neurofibromatosis 2 / genetics
  • Neuroma, Acoustic / genetics*
  • Point Mutation*
  • Polymerase Chain Reaction