Diagnosis and Management of Hereditary Meningioma and Vestibular Schwannoma

Recent Results Cancer Res. 2016:205:17-27. doi: 10.1007/978-3-319-29998-3_2.


Bilateral vestibular schwannomata and meningiomata are the tumours most commonly associated with neurofibromatosis type II (NF2). These tumours may also be seen in patients with schwannomatosis and familial meningioma, but these phenotypes are usually easy to distinguish. The main diagnostic challenge when managing these tumours is distinguishing between sporadic disease which carries low risk of subsequent tumours or NF2 with its associated morbidities and reduced life expectancy. This chapter outlines some of the diagnostic and management considerations along with associated evidence.

Keywords: Meningioma; NF2; Neurofibromatosis type II; Schwannomatosis; Vestibular schwannoma.

MeSH terms

  • Diagnosis, Differential
  • Genetic Predisposition to Disease
  • Genetic Testing
  • Humans
  • Meningeal Neoplasms / diagnosis*
  • Meningeal Neoplasms / genetics
  • Meningeal Neoplasms / therapy*
  • Meningioma / diagnosis*
  • Meningioma / genetics
  • Meningioma / therapy*
  • Neoplastic Syndromes, Hereditary / diagnosis*
  • Neoplastic Syndromes, Hereditary / genetics
  • Neoplastic Syndromes, Hereditary / therapy*
  • Neuroma, Acoustic / diagnosis*
  • Neuroma, Acoustic / genetics
  • Neuroma, Acoustic / therapy*
  • Risk Assessment