title: Prognostic value and management of spinal tumors in neurofibromatosis type 2 patients.
abstract: OBJECTIVE
  Neurofibromatosis type 2 (NF2) is a well-studied disease. Although spinal tumors are frequent, many issues concerning their prognosis and management still have to be clarified. The authors studied the clinical impact and radiological progression of spinal tumors in patients with NF2 to determine their prognostic value.
  
  
  METHODS
  A total of 80 NF2 patients were followed in the Lille University Hospital between 1987 and 2011. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both the location and size of each tumor were reported. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation.
  
  
  RESULTS
  The mean follow-up period was 8.8 years (range 1 to 24 years; SD: ±0.8), and the mean age at diagnosis was 27.2 years (range 6 to 73 years; SD: ±1.7). Among all patients, 48 harbored spinal tumors. Twenty of them were symptomatic, and 21 were operated on. Patients with spinal tumors had a lower age at diagnosis (p = 0.02), a higher number of intracranial meningiomas (p = 0.028) and schwannomas (p = 0.03), and more nonsense and frameshift mutations (p = 0.04).
  
  
  CONCLUSIONS
  Spinal tumors are common in NF2, and all patients should be regularly monitored by spinal MRI. The presence of spinal tumors seems to be a factor indicating poor prognosis. Clinical and radiological monitoring of spinal tumors could lead to early treatment both when clinical symptoms are present and in case of proven radiological evolution, maintaining a favorable functional prognosis as long as possible.
authors: Department of Neurosurgery, Lille University Hospital, rue E. Laine, 59037, Lille cedex, France. rabihdoc@hotmail.com
authors: Rabih
authors: R
authors: Aboukais
authors: Marc
authors: M
authors: Baroncini
authors: Fahed
authors: F
authors: Zairi
authors: Nicolas-Xavier
authors: NX
authors: Bonne
authors: Stéphane
authors: S
authors: Schapira
authors: Christophe
authors: C
authors: Vincent
authors: Jean-Paul
authors: JP
authors: Lejeune
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D018450:Disease Progression; D016515:Genes, Neurofibromatosis 2 / Q000502:physiology; D005838:Genotype; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D008579:Meningioma / Q000175:diagnosis* / Q000235:genetics; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D009442:Neurilemmoma / Q000175:diagnosis / Q000235:genetics; D016518:Neurofibromatosis 2 / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D010641:Phenotype; D011379:Prognosis; D012189:Retrospective Studies; D013125:Spinal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D055815:Young Adult