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原发性硬脊膜内髓外室管膜瘤:病例报告及文献复习

Primary intradural extramedullary ependymoma: case report and review of the literature.

作者信息

Duffau H, Gazzaz M, Kujas M, Fohanno D

机构信息

Departments of Neurosurgery 1 and Histology-Embryology-Cytogenetics, Hôpital de la Salpetrière, Paris, France.

出版信息

Spine (Phila Pa 1976). 2000 Aug 1;25(15):1993-5. doi: 10.1097/00007632-200008010-00021.

Abstract

STUDY DESIGN

The authors report the ninth case in the literature of a primary intradural extramedullary ependymoma of the spinal cord.

OBJECTIVE

To discuss surgical treatment and the physiopathologic hypothesis of this localization on the basis of the results of the present study and a review of the literature.

SUMMARY OF BACKGROUND DATA

Ependymoma is a glial tumor known to arise in the central nervous system. Intradural extramedullary location of this neoplasm has been exceptionally described previously.

METHODS

A 43-year-old woman was admitted to the authors' institution with an history of progressive paraplegia. Neurologic examination showed sensory loss below T1 and bladder disturbances. Magnetic resonance imaging revealed an enhanced thoracic intradural extramedullary tumor, extending from T1-T8. No other lesion in the central nervous system was found. Emergency surgical resection was performed.

RESULTS

Surgery gave confirmation of an encapsulated extramedullary tumor without attachment to the spinal cord or to the dura mater. Total removal was achieved under microscope. The postoperative course was uneventful, with complete neurologic recovery 3 months later. The patient has been well for 24 months of follow-up evaluation, without evidence of recurrence on magnetic resonance images. Histologic examination revealed the tumor as a benign ependymoma.

CONCLUSION

The encapsulated feature, the lack of attachment to the central nervous system, and the absence of other neoplastic processes within the brain or the spinal cord suggested that this lesion is a primary tumor developed from ectopic ependymal cells.

摘要

研究设计

作者报告了脊髓原发性硬脊膜内髓外室管膜瘤文献中的第九例病例。

目的

基于本研究结果及文献回顾,探讨该部位病变的手术治疗及病理生理假说。

背景资料总结

室管膜瘤是一种已知起源于中枢神经系统的神经胶质瘤。此前曾有过该肿瘤硬脊膜内髓外定位的罕见描述。

方法

一名43岁女性因进行性截瘫病史入住作者所在机构。神经系统检查显示T1以下感觉丧失及膀胱功能障碍。磁共振成像显示胸段硬脊膜内髓外肿瘤强化,从T1至T8。未发现中枢神经系统其他病变。进行了急诊手术切除。

结果

手术证实为包膜完整的髓外肿瘤,与脊髓或硬脑膜无粘连。在显微镜下实现了全切。术后过程顺利,3个月后神经功能完全恢复。在24个月的随访评估中患者情况良好,磁共振成像未显示复发迹象。组织学检查显示肿瘤为良性室管膜瘤。

结论

包膜完整的特征、与中枢神经系统无粘连以及脑或脊髓内无其他肿瘤性病变提示该病变是由异位室管膜细胞发展而来的原发性肿瘤。

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