Abstract

This study was conducted with the aim to estimate long-term tumor control and hearing preservation rates in patients with neurofibromatosis 2 (NF2)-related vestibular schwannoma (VS), document retreatment success rate, and assess the associated predictive factors. This was a retrospective analysis of patients with NF2-associated VS who underwent Gamma Knife radiosurgery (GKRS) between 2009 and 2020 and had a minimum follow-up of 1 year. Loss of tumor control was defined as greater than 10% increase in volume in more than one follow-up imaging or the need for retreatment in the form of repeat GKRS or surgery. The Kaplan-Meier method was used to evaluate actuarial tumor control and hearing preservation rates. In total, 85 patients with 133 VSs were included in the study. The mean age was 29.8 years. In total, 57 tumors showed tumor regression, 35 showed stable disease, and 23 progressed in size at last follow up. Actuarial tumor control rates after 1, 3, 5, and 9 years were 95%, 79%, 75%, and 55%, respectively, with overall tumor control rate being 85%. Hearing worsened in 39 patients, and facial nerve dysfunction occurred in 4 patients. Five tumors underwent retreatment with GKRS at a median duration of 27.6 months (19-36 months) following the first GKRS. This is the largest radiosurgical series of NF2-associated VS reported to date. GKRS provides a high rate of long-term local tumor control with a low risk of neurologic deprivation for patients with these tumors. The need for retreatment with GKRS, although low, is associated with good tumor control and lesser complications.

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