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Sporadic pediatric vestibular schwannoma: a case report in a 4-year-old boy

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Abstract

Sporadic vestibular schwannomas (VSs) are rare in children. When occurred in the pediatric population, they usually appear bilaterally and are related to neurofibromatosis type 2 (NF2). The current study reports a 4-year-old boy without family history of VS or NF2 who presented with a large (5.7-cm) VS involving the right cerebellopontine angle and internal auditory canal. Through seven-staged surgical interventions and two stereotactic γ‑knife radiosurgery, the disease was stabilized. At 2-year follow-up, the child had right ear hearing loss, grade IV facial palsy, and normal motor function and gait. No definite evidence of gene mutation regarding NF2 can be identified after sequence analysis and deletion/duplication testing. This case highlights the significance of considering the possibility of sporadic VSs, even in very young children. It emphasizes the importance of not overlooking initial symptoms, as they may indicate the presence of a large tumor and could potentially result in delayed diagnosis.

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No datasets were generated or analysed during the current study.

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Author information

Author notes

  1. Authors Kevin Li-Chun Hsieh and Tai-Tong Wong contributed equally to this work.

Authors and Affiliations

  1. Department of Medical Imaging, Taipei Medical University Hospital, Taipei City, Taiwan

    Cheng-Chieh Tsai & Kevin Li-Chun Hsieh

  2. Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei City, Taiwan

    Chia-Lang Fang

  3. Department of Pathology, Taipei Medical University Hospital, Taipei Medical University, Taipei City, Taiwan

    Chia-Lang Fang

  4. Pediatric Brain Tumor Program, Taipei Cancer Center and Taipei Neurological Institute, Taipei Medical University, Taipei City, Taiwan

    Minhua Liao, Kevin Li-Chun Hsieh & Tai-Tong Wong

  5. Division of Pediatric Neurosurgery, Department of Neurosurgery, Taipei Medical University Hospital, Taipei City, Taiwan

    YiShan Yang & Tai-Tong Wong

  6. Department of Radiology, School of Medicine, College of Medicine, Taipei Medical University, Taipei City, 110301, Taiwan

    Kevin Li-Chun Hsieh

  7. Graduate Institute of Clinical Medicine, College of Medicine, Taipei Medical University, Taipei City, Taiwan

    Tai-Tong Wong

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Contributions

KH and TTW conceptualized and designed the study. KH, TTW, CLF, and ML analyzed and interpreted the data. CLF and ML performed the sample processing, extracting, and sequencing experiments. CCT, CLF, and KH drafted the initial manuscript. CCT, YY, KH, and TTW reviewed and revised the manuscript. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.

Corresponding author

Correspondence to Kevin Li-Chun Hsieh.

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Tsai, CC., Fang, CL., Liao, M. et al. Sporadic pediatric vestibular schwannoma: a case report in a 4-year-old boy. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06398-5

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