基础医学与临床 ›› 2012, Vol. 32 ›› Issue (8): 956-959.

• 临床园地 • 上一篇    下一篇

原发椎管内外周型原始神经外胚层肿瘤2例

曲彦明,张宏伟,张明山,于春江   

  1. 首都医科大学第十一临床医学院,北京三博脑科医院
  • 收稿日期:2012-06-11 修回日期:2012-06-21 出版日期:2012-08-05 发布日期:2012-07-17
  • 通讯作者: 于春江 E-mail:CJyu1955@sina.com

Primary intraspinal peripheral primitive neuroectodermal tumor: two cases report

  • Received:2012-06-11 Revised:2012-06-21 Online:2012-08-05 Published:2012-07-17

摘要: 摘要:目的 探讨椎管内原发外周型原始神经外胚层肿瘤的临床特点、诊断、病理表现和治疗进展。方法 分析2例经影像学、手术和病理检测证实的椎管内原始神经外胚层肿瘤,回顾相关文献,总结其临床特点。结果 2例病变术前行MRI检查示等T1WI、T2WI信号,增强后明显强化;镜下部分切除病变,术后病理检查提示均为外周型原始神经外胚层肿瘤,术后行放疗和化疗,随访。结论 椎管内原始神经外胚层肿瘤发病率低,多发生于腰骶部,以外周型常见,病程短,预后差,病理结果可指导术后放化疗方案,新的诊疗策略有望提高患者生存率。

关键词: 关键词:原始神经外胚层肿瘤, 椎管

Abstract: Abstract: Objective To investigate the clinical characteristics, diagnosis, pathological features and treatment of primary intraspinal peripheral primitive neuroectodermal tumor (pPNET). Methods The clinical materials of 2 intraspinal PNET,such as image data,neurosurgical treatment and histopathology features were analyzed retrospectively. Results Two PNETs were isointense on T1-weighted images and T2-weighted images, and with Gd-DTPA enhancement apparently. Both of them were treated by surgery and underwent subtotal resection. They were identified peripheral primitive neuroectodermal tumor by histopathological examination and immunohistochemical staining. Furthermore, they underwent radiotherapy and chemotherapy. Both of them were followed up. Conclusions Intraspinal PNET is very rare and frequently located at lumbar/lumbosacral spinal level and most of them are peripheral PNET. The patient has short duration of symptoms and poor prognosis. Treatment proposals are based on histopathological results. Newer therapeutic strategy will improve the survival of patient.

Key words: Key words: primitive neuroectodermal tumor, intraspinal

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