Daisuke Hirokawa1, Kenichi Usami1, Sukwoo Hong1, Hideki Ogiwara2. 1. Division of Neurosurgery, National Center for Child Health and Development, Okura 2-10-1, Setagaya-ku, Tokyo, 157-8535, Japan. 2. Division of Neurosurgery, National Center for Child Health and Development, Okura 2-10-1, Setagaya-ku, Tokyo, 157-8535, Japan. hideki_o@d5.dion.ne.jp.
Abstract
INTRODUCTION: Pediatric schwannomas are rare, and most of them are associated with neurofibromatosis type 2 (NF2) and usually located in the vestibular nerve. Herein, we present the first pediatric case of intracranial schwannoma derived from the IX/X nerve complex unrelated to NF2. CASE REPORT: The patient was a 9-year-old boy who presented with a 3-month history of headache and nausea. There was no family history of NF2. Imaging studies revealed a cystic lesion with enhanced wall in the left cerebellomedullary fissure. During the operation, the IX/X nerve complex was strongly adhered to the tumor at the jugular foramen. The tumor was totally excised, and the postoperative MRI demonstrated no residual tumor. Histopathological diagnosis was schwannoma. Genetic analysis revealed no mutation associated with NF2 and schwannomatosis. CONCLUSION: We reported the first case of pediatric lower cranial nerve schwannoma which was not associated with NF2. The schwannoma should be included as differential diagnosis of pediatric posterior fossa tumors.
INTRODUCTION: Pediatric schwannomas are rare, and most of them are associated with neurofibromatosis type 2 (NF2) and usually located in the vestibular nerve. Herein, we present the first pediatric case of intracranial schwannoma derived from the IX/X nerve complex unrelated to NF2. CASE REPORT: The patient was a 9-year-old boy who presented with a 3-month history of headache and nausea. There was no family history of NF2. Imaging studies revealed a cystic lesion with enhanced wall in the left cerebellomedullary fissure. During the operation, the IX/X nerve complex was strongly adhered to the tumor at the jugular foramen. The tumor was totally excised, and the postoperative MRI demonstrated no residual tumor. Histopathological diagnosis was schwannoma. Genetic analysis revealed no mutation associated with NF2 and schwannomatosis. CONCLUSION: We reported the first case of pediatric lower cranial nerve schwannoma which was not associated with NF2. The schwannoma should be included as differential diagnosis of pediatric posterior fossa tumors.
Entities:
Keywords:
Lower cranial nerves; Neurofibromatosis type 2; Pediatric intracranial schwannoma
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