椎管内原发性原始神经外胚瘤的诊断与治疗

目的探讨椎管内原始神经外胚瘤（PNET）的诊断和治疗方法，以提高其早期诊断和合理治疗的水平，改善患者的预后。方法根据肿瘤侵袭部位不同，5例椎管内原始神经外胚瘤首先给予后正中入路或与经胸联合入路的手术切除，术后分别给予不同疗程的局部放疗和全身化疗。结果常规及免疫组化病理检查使PNET得以确诊；5例患者除1例生存至今33个月外，其余虽经综合治疗，仍相继出现原位和（或）胸腔、肝脏、肺部等转移，分别在术后14～20个月死亡。结论MRI检查有助于椎管内PNET定位诊断，确切的诊断有赖于病理学检查；及早手术是其首选治疗，辅助放疗和化疗对预防和延缓PNET的复发有益。目前椎管内PNET患者预后差，有效的治疗新方法有待进一步研究。

Diagnosis and treatment of spinal primitive neuroectodermal tumor

Objective To evaluate the diagnosis and treatment of spinal primitive neuroectodermal tumor （PNET） with an aim to provide a proper therapy and improve the survival in these patients. Methods Five patients with spinal PNET received tumor＇s gross total resection by posterior or combined with thoracic approach and were followed by chemotherapy and local radiotherapy, Results The tumors were confirmed by immunohistochemical pathology, Four of five patients of spinal PNET died within 14 - 20 months postoperation, caused by rapid recurrence in spinal or/and thoracic cavity, hung, and hepatic metastasis. Only one has been healthy for 33 months since surgery. Conclusion MRI is helpful for the diagnosis of PNET location, while the timber diagnosis relies on the patnological examination, The tumor＇s resection should be prominent and chemotherapy and neuraxis radiation may offer hopes for long term and good quality survival. As poor prognosis in the PNET patients, the new treatment strategies should be concerned in further study.