The Japanese NF2 population has heterogeneous clinical features. Risk factors for progressive disability include younger age of onset, positive family history, positive treatment history, and specific neurologic deficits.
We conducted a feasibility study to investigate the therapeutic effect of bevacizumab on vestibular schwannomas (VS) associated with neurofibromatosis type 2 (NF2) in a sample of Japanese patients. Ten NF2 patients were selected between 2013 and 2018: nine women and one man, with ages ranging from 12 to 45 years (mean: 29.4). Bevacizumab was administered intravenously in 5 mg/kg doses four times, with an inter-dose interval of 2 weeks. Seventeen tumors were followed for 3-72 months (mean: 39). A reduction from baseline tumor volume of at least 20% was considered a therapeutic radiologic response. Maximum reduction in tumor volume was identified in the 3rd month in 11 tumors, and in the 6th month in three tumors. Three tumors did not show any response to bevacizumab. A radiologic response was detected in seven tumors (41%). There was a significantly lower tumor volume mean in the 3rd month in comparison to the baseline for the entire sample. Tumors in patients aged 25 and above showed a significant reduction in volume in the 3rd month and significantly lower tumor-volume-to-baseline ratio than younger patients in both the 3rd and 6th months. The interaction between 'time' and 'age group' factors significantly affected the therapeutic outcome of bevacizumab on tumor volume. This study investigated the therapeutic effects of bevacizumab on NF2-associated vestibular schwannomas in Japanese patients. Bevacizumab appears to be a useful therapeutic choice in NF2 cases to control the growth of VS. Therefore, a randomised control trial to prove this assumption is necessary.
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