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.
Keywords: Auditory brainstem implant, bevacizumab, cochlear implant, cerebellopontine angle.
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