ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2020
Managing NF2-associated vestibular schwannomas in children and young adults: review of an institutional series regarding effects of surgery and bevacizumab on growth rates, tumor volume, and hearing quality.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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11 citing papers in PubMed, 16 citations in OpenAlex.
- Targeted Medical Therapy for Vestibular Schwannomas: Evidence, Limits, and Future Directions-A Scoping Review.Current issues in molecular biology · 2026Review
- Gene therapy and genome-editing for schwannoma in NF2-related schwannomatosis: current understanding and future directions.Journal of neuro-oncology · 2025Review
- Management Strategies of Neurofibromatosis Type 2 in Pediatric Patients : Challenges and Emerging Therapies.Journal of Korean Neurosurgical Society · 2025Article
- Spatial mapping of immune cell environments in NF2-related schwannomatosis vestibular schwannoma.Nature communications · 2025Article
- Vestibular Schwannoma and Tinnitus: A Systematic Review of Microsurgery Compared to Gamma Knife Radiosurgery.Journal of clinical medicine · 2024Review
- Neurosurgical Treatment and Outcome of Pediatric Skull Base Lesions: A Case Series and Review of the Literature.Children (Basel, Switzerland) · 2023Article
- Intracranial paragangliomas versus schwannomas: Role of dynamic susceptibility contrast perfusion and diffusion MRI.Journal of neuroimaging : official journal of the American Society of Neuroimaging · 2022Article
- Article
- NF2-Related Schwannomatosis Exhibiting the Complete Constellation of Multiple Inherited Schwannomas, Meningiomas, and Ependymomas (MISME Syndrome).Case reports in neurologyArticle
- Withdrawal of bevacizumab is associated with rebound growth of vestibular schwannomas in neurofibromatosis type 2-related schwannomatosis patients.Neuro-oncology advancesArticle
- Clinical trials targeting neurofibromatoses-associated tumors: a systematic review.Neuro-oncology advancesReview
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Authors and funding
7 authors at 2 institutions in 1 country.
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Abstract
We reviewed our experience in managing of NF2-associated vestibular schwannoma (VS) in children and young adults regarding the effect of surgery and postoperative bevacizumab treatment. A total of 579 volumetric and hearing data sets were analyzed. The effect of surgery on tumor volume and growth rate was investigated in 46 tumors and on hearing function in 39 tumors. Long-term hearing follow-up behavior was compared with 20 non-operated ears in additional 15 patients. Sixteen operated VS were treated with bevacizumab. Mutation analysis of the NF2 gene was performed in 25 patients. Surgery significantly slowed down VS growth rate. Factors associated with a higher growth rate were increasing patient age, tumor volume, and constitutional truncating mutations. Immediately after surgery, functional hearing was maintained in 82% of ears. Deterioration of hearing was associated with initial hearing quality, larger tumor volumes, and larger resection amounts. Average hearing scores were initially better in the group of non-operated VS. Over time, hearing scores in both groups worsened with a similar dynamic. During bevacizumab treatment of residual tumors, four different patterns of growth were observed. Decompression of the internal auditory canal with various degrees of tumor resection decreases the postoperative tumor growth rates. Carefully tailored BAEP-guided surgery does not cause additional hearing deterioration. Secondary bevacizumab treatment showed heterogenous effects both regarding tumor size and hearing preservation. It seems that postoperative tumor residuals, that grow slower, behave differently to bevacizumab than reported for not-operated faster growing VS.
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