Hirokawa Daisuke, Usami Kenichi, Hong Sukwoo, Ogiwara Hideki
Division of Neurosurgery, National Center for Child Health and Development, Okura 2-10-1, Setagaya-ku, Tokyo, 157-8535, Japan.
Childs Nerv Syst. 2019 Jun;35(6):1041-1044. doi: 10.1007/s00381-018-04045-4. Epub 2019 Jan 13.
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.
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.
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.
小儿神经鞘瘤较为罕见,其中大多数与2型神经纤维瘤病(NF2)相关,且通常位于前庭神经。在此,我们报告首例源自第IX/X神经复合体且与NF2无关的小儿颅内神经鞘瘤病例。
该患者为一名9岁男孩,有3个月的头痛和恶心病史。无NF2家族史。影像学检查显示左小脑延髓裂有一个壁强化的囊性病变。手术过程中,第IX/X神经复合体在颈静脉孔处与肿瘤紧密粘连。肿瘤被完全切除,术后MRI显示无残留肿瘤。组织病理学诊断为神经鞘瘤。基因分析未发现与NF2和神经鞘瘤病相关的突变。
我们报告了首例与NF2无关的小儿低位颅神经神经鞘瘤病例。该神经鞘瘤应作为小儿后颅窝肿瘤的鉴别诊断之一。