TY - JOUR
T1 - Sporadic pediatric vestibular schwannoma
T2 - a case report in a 4-year-old boy
AU - Tsai, Cheng Chieh
AU - Fang, Chia-Lang
AU - Liao, Minhua
AU - Yang, Yi Shan
AU - Hsieh, Kevin Li-Chun
AU - Wong, Tai Tong
N1 - Publisher Copyright:
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2024.
PY - 2024/7
Y1 - 2024/7
N2 - 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.
AB - 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.
KW - Acoustic neuroma
KW - Neurofibromatosis type 2
KW - Neuroma
KW - Pediatric
KW - Pediatric neurosurgery
KW - Sporadic
KW - Vestibular schwannoma
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U2 - 10.1007/s00381-024-06398-5
DO - 10.1007/s00381-024-06398-5
M3 - Article
C2 - 38709258
AN - SCOPUS:85192176401
SN - 0256-7040
VL - 40
SP - 2251
EP - 2255
JO - Child's Nervous System
JF - Child's Nervous System
IS - 7
ER -