Aurore Picry1,2, Nicolas-Xavier Bonne3, Juliette Ding, Rabih Aboukais4, Jean-Paul Lejeune4, Marc Baroncini4, Frédérique Dubrulle5, Christophe Vincent3. 1. Department of Otology and Neurotology, Roger Salengro Hospital, Lille University Hospital, Lille, France. aurore.picry@chru-lille.fr. 2. Department of Anatomy, "Henri Warembourg" Faculty of Medicine, University of Lille, Lille, France. aurore.picry@chru-lille.fr. 3. Department of Otology and Neurotology, Roger Salengro Hospital, Lille University Hospital, Lille, France. 4. Department of Neurosurgery, Roger Salengro Hospital, Lille University Hospital, Lille, France. 5. Department of Radiology, Claude Huriez Hospital, Lille University Hospital, Lille, France.
Abstract
OBJECTIVES/HYPOTHESIS: To determine the long-term growth rate of vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2) patients based on volumetric measurements. STUDY DESIGN: Retrospective review. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:2358-2362, 2016.
OBJECTIVES/HYPOTHESIS: To determine the long-term growth rate of vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2) patients based on volumetric measurements. STUDY DESIGN: Retrospective review. METHODS: 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. RESULTS: 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). CONCLUSIONS: This study helps to assess the long-term growth profile of VS in a population of NF2patients with untreated VS. These data could help to better acknowledge VS natural growth history. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:2358-2362, 2016.
Authors: Daniel Moualed; Jonathan Wong; Owen Thomas; Calvin Heal; Rukhtam Saqib; Cameron Choi; Simon Lloyd; Scott Rutherford; Emma Stapleton; Charlotte Hammerbeck-Ward; Omar Pathmanaban; Roger Laitt; Miriam Smith; Andrew Wallace; Mark Kellett; Gareth Evans; Andrew King; Simon Freeman Journal: Eur J Hum Genet Date: 2022-01-24 Impact factor: 4.246