title: Long-term growth rate of vestibular schwannoma in neurofibromatosis 2: A volumetric consideration.
abstract: To determine the long-term growth rate of vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2) patients based on volumetric measurements.
  
  
  
  Retrospective review.
  
  
  
  We retrospectively reviewed magnetic resonance imaging (MRI) scans acquired from patients with NF2 from 1999 to 2013. Patients with an annual radiologic follow-up over at least 3 consecutive years were included. Volumetric VS growth was prospectively measured using a three-dimensional imaging workstation and through manual contouring of the lesion. Time to tumor progression was assessed according to the Response Evaluation in Neurofibromatosis and Schwannomatosis Tumor Measurement Group.
  
  
  
  Eighteen patients presenting with a total of 26 VSs were included. The mean age at diagnosis was 26.1 years (range, 7 to 49 years). One hundred five MRI scans were analyzed during a median radiological follow-up of 5.6 years (range, 3 to 12 years). The annual volume and diameter growth rates were respectively 218.3 mm(3) /yr (range, -10 to 1,250 mm(3) /yr) and 0.9 mm/yr (range, -0.5 to 4.5 mm/yr). Time to tumor progression was 3 years (median survival). There was a weak correlation between volumetric and linear measurements (P < .0001, linear regression, n = 26, r(2) = 0.58). Among the 26 VSs, 76.9% (20/26) showed progression (VS growth more than 20%), 19.2% were stable, and 3.9% (1/26) exhibited shrinkage (spontaneous regression of more than 20% of the initial volume).
  
  
  
  This study helps to assess the long-term growth profile of VS in a population of NF2 patients with untreated VS. These data could help to better acknowledge VS natural growth history.
  
  
  
  4 Laryngoscope, 126:2358-2362, 2016.
authors.affiliation: Department of Otology and Neurotology, Roger Salengro Hospital, Lille University Hospital, Lille, France. aurore.picry@chru-lille.fr.|Department of Anatomy, "Henri Warembourg" Faculty of Medicine, University of Lille, Lille, France. aurore.picry@chru-lille.fr.
authors.forename: Aurore
authors.initials: A
authors.lastname: Picry
authors.affiliation: Department of Otology and Neurotology, Roger Salengro Hospital, Lille University Hospital, Lille, France.
authors.forename: Nicolas-Xavier
authors.initials: NX
authors.lastname: Bonne
authors.forename: Juliette
authors.initials: J
authors.lastname: Ding
authors.affiliation: Department of Neurosurgery, Roger Salengro Hospital, Lille University Hospital, Lille, France.
authors.forename: Rabih
authors.initials: R
authors.lastname: Aboukais
authors.affiliation: Department of Neurosurgery, Roger Salengro Hospital, Lille University Hospital, Lille, France.
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Lejeune
authors.affiliation: Department of Neurosurgery, Roger Salengro Hospital, Lille University Hospital, Lille, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Baroncini
authors.affiliation: Department of Radiology, Claude Huriez Hospital, Lille University Hospital, Lille, France.
authors.forename: Frédérique
authors.initials: F
authors.lastname: Dubrulle
authors.affiliation: Department of Otology and Neurotology, Roger Salengro Hospital, Lille University Hospital, Lille, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Vincent
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D021621:Imaging, Three-Dimensional; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000150:complications* / Q000473:pathology; D009464:Neuroma, Acoustic / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology*; D011859:Radiography; D012189:Retrospective Studies; D013997:Time Factors; D047368:Tumor Burden; D055815:Young Adult
keywords: Neurofibromatosis type 2; growth rate; magnetic resonance imaging; vestibular schwannoma; volumetric