椎管内原始神经外胚层肿瘤

目的探讨原发于椎管内的原始神经外胚层肿瘤（PNET）的诊断与治疗。方法回顾6例椎管内PNET的临床资料，分析其影像学、病理学特点及治疗方法。结果6例椎管内肿瘤有3例化于硬脊膜下，1例局限于硬膜外，1例自椎间孔由硬膜外生长至椎管外，1例累及髓内、椎体、椎旁软组织。全部病例行手术治疗，全切除2例，近全切除2例，活检2例。术后均行HE染色及免疫组化病理检查。术后随访的5例患者中单纯放疗2例，单纯化疗1例，放化疗均进行共3例，均存活。结论椎管内PNET影像学表现多样，诊断困难。病理学检查是诊断的依据。手术切除范围可根据肿瘤生长特点灵活掌握。放化疗对肿瘤有效。但尚无统一的治疗方法。

Intraspinal primitive neuroectodermal tumor

Objective To investigate the diagnosis and treatment of primary intraspinal primitive neuroectodermal tumor (PNET). Methods The clinical materials of 6 intraspinal PNET, such as image data, histopathology features, methods of treatment, were analyzed retrospectively. Results 3 tumors were localized in intradural area, 1 in epidural area, 1 grew from epidural to extraspinal area, 1 involved intramedullary location, vertebra and paravertebral soft tissue. All cases were treated by surgery, 2 cases underwent total resection and 2 cases subtotal resection, 2 cases underwent biopsy. HE and immunohistochemical staining were performed in every case. Among the 5 cases who had been followed up, 2 underwent single radiotherapy, 2 underwent single chemotherapy and 3 underwent both of the two ways. All of the 5 cases survived. Conclusions The image features of intraspinal PNET are variform, the diagnosis is difficult. Histopathology is the evidence of diagnosis. The range of resection is decided according to the growth character of tumor. Radiotherapy and chemotherapy are effective. There is no uniform method of treatment.