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

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

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。

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 subtotal resection.The tumors were confirmed by convention and immunohistochemical pathology.All cases were followed by chemotherapy and local radiotherapy.1 case died within 16 months post-operation,1 case losed following up within 14 months,1 case has been healthy for 16 months since surgery.Conclusion MRA is helpful for the diagnosis and location of PNET,while the further diagnosis relies on the pathological examination.The tumor total resection should be prominent and chemotherapy and neuraxis radiotherapy may offer hopes for long term survival and good life quality.