title: Intracranial meningiomas and neurofibromatosis type 2.
abstract: OBJECTIVE
  In spite of the few clinical studies regarding the occurrence of intracranial meningiomas, their prognosis in neurofibromatosis type 2 (NF2) has not been accurately assessed and their management remains controversial. This study aims to compare NF2 patients with intracranial meningiomas to those without, and consequently to identify prognostic factors in attempt to improve the management of these tumors.
  
  
  METHODS
  This retrospective study includes a total of 80 NF2 patients followed at Lille Hospital Center between 1987 and 2011. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both location and size of each tumor were reported.
  
  
  RESULTS
  The mean follow-up period was 8.8 years (range 1-24 years; SD: ±0.8) and the mean age at diagnosis was 27.2 years (range 6-73 years; SD: ±1.7). Among all patients, 34 harbored intracranial meningiomas. Patients with intracranial meningiomas had a higher number of intracranial schwannomas, spinal tumors and cutaneous tumors (p < 0.05). They underwent more surgical procedures (p < 0.012). Twenty five intracranial meningiomas were surgically removed in 17 patients. The decision to perform surgery was taken in 10 cases for symptomatic tumors and in 15 cases for growing asymptomatic tumors determined by radiology. The histological analysis found a high rate of fibroblastic, transitional or grade 2 meningiomas preferentially located at the cerebri falx.
  
  
  CONCLUSIONS
  Intracranial meningiomas are common in NF2. They are associated with poor prognosis factors. Clinical and radiological monitoring could lead to early treatment of these tumors both when clinical symptoms are present and in case of proven radiological evolution, and thus trying to maintain a favorable functional prognosis for as long as possible.
authors.affiliation: Department of Neurosurgery, Lille University Hospital, rue E. Laine, 59037, Lille cedex, France. rabihdoc@hotmail.com
authors.forename: Rabih
authors.initials: R
authors.lastname: Aboukais
authors.forename: Fahed
authors.initials: F
authors.lastname: Zairi
authors.forename: Marc
authors.initials: M
authors.lastname: Baroncini
authors.forename: Nicolas-Xavier
authors.initials: NX
authors.lastname: Bonne
authors.forename: Stéphane
authors.initials: S
authors.lastname: Schapira
authors.forename: Christophe
authors.initials: C
authors.lastname: Vincent
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Lejeune
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D016515:Genes, Neurofibromatosis 2 / Q000502:physiology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008579:Meningioma / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D016518:Neurofibromatosis 2 / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D011379:Prognosis; D012189:Retrospective Studies; D013125:Spinal Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D055815:Young Adult