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Updated: Jul 15, 2026
Primary Culture of Human Vestibular Schwannomas
Published on: July 20, 2014
Current Management of Large Vestibular Schwannomas for NF2 Patients in a National Reference Center
Pierre-Cyril Comes1, Matthieu Peyre1,2, Marc Sanson2,3
1Neurosurgical Department, Pitié-Salpétrière Hospital, Assistance Publique Hôpitaux de Paris, Paris, France.
Objective:
Recently, treatment decision making for large vestibular schwannomas (VS) in patients with neurofibromatosis type 2 (NF2) has become increasingly challenging due to the availability of multiple therapeutic options including surgery, bevacizumab (an anti-VEGF), radiosurgery, and observation; and it often remains an arbitrary decision based on local practices without firm recommendations. Our objective is to discuss the multimodal treatment options for Koos IV VS in a national reference center for NF2.
Study Design:
Single-institution retrospective cohort study.
Methods:
All NF2 patients with Koos IV VS who visited our center, the National Reference Center for NF2 Rare Disease in Pitié-Salpétrière Hospital of Paris, between January 2016 and December 2018 were included. Clinical charts, radiology, operative reports, and audiograms were reviewed.
Results:
Among 54 NF2 patients with Koos IV VS (mean maximum extrameatal diameter: 34 mm; range:17-62 mm), 27 were operated on for 28 VS; 21 were treated with bevacizumab; and six were observed. In the surgical group, VS resections were gross total, near-total, subtotal, or partial in 32%, 25%, 32%, and 11%, respectively; and a good (House-Brackmann grades I-II) facial nerve function was achieved in 81.5% at 1 year. Hearing was preserved in 14%, 78%, and 66% of the surgical (n = 7), bevacizumab (n = 9), and observation (n = 3) patients, respectively.
Conclusion:
All therapeutic options, including surgery and/or bevacizumab and occasionally observation, should be proposed to NF2 patients with large VS in the setting of dedicated centers. A decision-making tree is proposed for Koos IV VS management based on tumor evolution, hearing and clinical status of the patient, and contralateral VS size.
Level Of Evidence:
4, case series study, historically controlled study Laryngoscope, 131:E98-E107, 2021.
Insights
Treatment for large vestibular schwannomas (VS) in neurofibromatosis type 2 (NF2) patients involves surgery, bevacizumab, or observation. A decision-making tree is proposed for managing Koos IV VS in specialized centers.
Area of Science:
- Neuro-oncology
- Otolaryngology
- Genetics
Background:
- Management of large vestibular schwannomas (VS) in neurofibromatosis type 2 (NF2) patients is complex due to multiple treatment options.
- Current decisions often lack firm recommendations, relying on local practices.
Purpose of the Study:
- To discuss multimodal treatment strategies for Koos IV VS in NF2 patients.
- To propose a decision-making framework for managing large VS in a national reference center.
Main Methods:
- Retrospective cohort study of NF2 patients with Koos IV VS (January 2016 - December 2018).
- Review of clinical charts, radiology, operative reports, and audiograms.
- Analysis of outcomes for surgery, bevacizumab, and observation groups.
Main Results:
- 54 NF2 patients with Koos IV VS were analyzed.
- Treatment distribution: 27 surgery, 21 bevacizumab, 6 observation.
- Good facial nerve function (81.5%) achieved post-surgery; hearing preservation varied (14% surgery, 78% bevacizumab, 66% observation).
Conclusions:
- Multimodal options (surgery, bevacizumab, observation) should be offered to NF2 patients with large VS.
- A decision-making tree is proposed based on tumor evolution, hearing, and patient status.
