High-Grade Glioma is not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient

Neurosurgery. 2018 Aug 1;83(2):193-196. doi: 10.1093/neuros/nyx374.

Abstract

Background: The Manchester criteria for neurofibromatosis type 2 (NF2) include a range of tumors, and gliomas were incorporated in the original description. The gliomas are now widely accepted to be predominantly spinal cord ependymomas.

Objective: To determine whether these gliomas include any cases of malignant glioma (WHO grade III and IV) through a database review.

Methods: The prospective database consists of 1253 patients with NF2. 1009 are known to be alive at last follow-up.

Results: There was a single case of glioblastoma multiforme (GBM; World Health Organization grade IV) in the series and no WHO grade III gliomas. The GBM was in a patient who had previously undergone stereotactic radiosurgery for a vestibular schwannoma.

Conclusion: High-grade gliomas are not a feature of NF2 in the unirradiated patient and should be excluded from the diagnostic criteria.

MeSH terms

  • Adult
  • Female
  • Glioma / epidemiology
  • Glioma / etiology*
  • Humans
  • Male
  • Middle Aged
  • Neurofibromatosis 2 / complications*
  • Neurofibromatosis 2 / radiotherapy
  • Prospective Studies
  • Radiosurgery / adverse effects
  • Young Adult