椎管内外周性原始神经外胚层肿瘤的诊断与治疗并文献复习

目的探讨原发于椎管内的外周性原始神经外胚层肿瘤（pPNET）的诊断与治疗方法。方法结合文献回顾性分析3例椎管内pPNET的临床资料,分析其影像学、病理特点、治疗方法及预后。结果2例完全位于椎管内硬膜外,1例自椎管内硬膜外经椎间孔累及相应椎体及同侧腰大肌。全部经手术治疗,全切除2例,大部切除1例。术后均经常规病理及免疫组化确诊,术后给与不同程度的局部放疗和全身化疗。1例14个月失访,1例16个月后死亡,1例生存至今。结论pPNET发病急促,MR有助于椎管内定位,免疫组化有助于定性诊断。首选及早手术,术中尽可能全切病灶,术后综合放化疗对提高生存有益。

Diagnosis and treatment of spinal peripheral primitive neuroectodermal tumor:with literature review

Objective To evaluate the diagnosis and treatment of spinal peripheral primitive neuroectodermal tumor(pPNET) with an aim to provide a proper therapy.Methods The clinical material of 3 intraspinal pPNET,such as image data,histopathology features,prognosis and methods of treatment were analyzed retrospectively.Results 2 cases were localized in epidural,1 cases grew from epidural to extraspinal area,vertebral body and psoas major.All cases were treated by surgery,2 cases underwent total resection and 1 case s...