椎管内原始性神经外胚瘤(附四例报告)

目的 探讨椎管内原始性神经外胚瘤的临床特点及分型、诊断、病理特点及治疗.方法 4例经影像学、手术和病理学证实的椎管内原始性神经外胚瘤,并回顾相关文献,总结所报告病例.结果 3例显微镜下全切,1例大部切除;术后病理检查提示中枢性原始性神经外胚瘤2例,外周性原始性神经外胚瘤2例;术后3例均给予全神经轴放疗和全身化疗,1例因经济原因术后出院后未再接受其他治疗;2例随访10个月及14个月仍生存,1例随访25个月死亡,1例随访6个月后失访.结论 椎管内原始性神经外胚瘤多见于成人;病程短;预后差;应根据病理分型制定手术后放化疗方案;CD99可作为临床上区别中枢性原始性神经外胚瘤和外周性原始性神经外胚瘤的免疫组化指标.

4 cases of intraspinal primitive neuroectodermal tumor

Objective To investigate the diagnosis, patterns, pathology and treatment of primary intraspinal primitive neuroectodermal tumor (PNET). Methods The clinical materials of 4 intraspinal PNET, such as image data, histopathoiogy features, methods of treatment, were analyzed retrospectively. Results All cases were treated by surgery, 3 cases underwent total resection and 1 cases subtotal resection. 2 central primitive neuroectodermal tumors and 2 peripheral primitive neuroectodermal tumors were revealed by histopathological examination and immunohistochemical staining. Among the 4 cases who had been followed up, 3 underwent radiotherapy and chemotherapy, 1 did not receive any adjuvant therapy due to socioeconomic issues. Conclusions The age of intraspinal PNET is much older than the average age of manifestation for intracranial PNET. The history of intraspinal PNET is short. It is important that chemotherapy plans for cPNET are different from those for pPNET; Expression of CD99 offers an easy way of making a differential diagnosis between the central and peripheral PNET.