Current Management of Large Vestibular Schwannomas for NF2 Patients in a National Reference Center

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 Pitie-Salpetriere 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.