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儿童脊髓脊索样脑膜瘤:一例报告并文献复习

Spinal chordoid meningioma in a child: A case report and review of the literature.

作者信息

Wu Liang, Yang Tao, Fang Jingyi, Zhang Junting, Xu Yulun

机构信息

Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, P.R. China.

Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing 100050, P.R. China.

出版信息

Oncol Lett. 2015 Dec;10(6):3727-3731. doi: 10.3892/ol.2015.3765. Epub 2015 Sep 29.

Abstract

As an uncommon subtype of meningioma, chordoid meningioma (CM) of the spinal canal is extremely rare. There have been only two reported cases of intraspinal CM in the literature, and this lesion has not been previously reported in the pediatric age group. To the best of our knowledge, the present study reports the first case of spinal chordoid meningioma in a pediatric patient. A 12-year-old female presented with a 3-month history of progressive numbness and weakness in the right-side limbs, and intermittent pain in the neck and right shoulder. Spinal magnetic resonance imaging (MRI) revealed an intraspinal lesion at the C2-3 level with irregularly heterogeneous enhancement. The patient underwent a C2-3 laminotomy. Due to adhesion to the dura and proximity to the vertebral artery, the tumor was partially removed intraoperatively. The post-operative course was uneventful and the symptoms were apparently relieved. The patient experienced recurrence 5 years subsequent to surgery. MRI revealed an extradural regrown tumor at the C2-5 level. Partial removal combined with radiotherapy was performed. However, the patient experienced progression of tetraplegia and succumbed to severe pneumonia and respiratory failure 5 months subsequent to the second surgery. In the present study, the clinicoradiological findings and treatment outcome of this rare entity are discussed, in addition to a review of the relevant literature. Spinal CMs should be included in the differential diagnosis of intraspinal tumors of the pediatric spine. Multidisciplinary treatment, consisting of total surgical removal and adjuvant radiotherapy, should be considered due to the aggressive nature of this abnormality and the risk of long-term recurrence.

摘要

作为脑膜瘤的一种罕见亚型,椎管内脊索样脑膜瘤(CM)极为罕见。文献中仅报道过2例椎管内CM病例,且此前未见该病变在儿童年龄组中的报道。据我们所知,本研究报告了首例小儿脊髓脊索样脑膜瘤病例。一名12岁女性,有右侧肢体进行性麻木和无力3个月病史,伴有颈部和右肩部间歇性疼痛。脊髓磁共振成像(MRI)显示C2 - 3水平有一椎管内病变,增强扫描呈不规则不均匀强化。患者接受了C2 - 3椎板切开术。由于肿瘤与硬脑膜粘连且靠近椎动脉,术中仅部分切除肿瘤。术后过程顺利,症状明显缓解。患者术后5年复发。MRI显示C2 - 5水平硬膜外肿瘤复发。进行了部分切除并联合放疗。然而,患者在第二次手术后5个月出现四肢瘫痪进展,最终死于严重肺炎和呼吸衰竭。在本研究中,除了回顾相关文献外,还讨论了这种罕见疾病的临床放射学表现和治疗结果。脊髓CM应纳入小儿脊柱椎管内肿瘤的鉴别诊断。鉴于该病变的侵袭性和长期复发风险,应考虑采用包括手术全切和辅助放疗在内的多学科治疗。

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