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目的:探讨颅内幕上原始神经外胚叶肿瘤的临床特征、手术治疗及预后。方法:本文回顾性分析了南京医科大学第一附属医院2000～2008年间收治的经手术及病理证实的幕上原始神经外胚叶肿瘤病例共6例,通过分析每个病例的临床资料,对该病的临床特点、手术治疗效果及预后进行了总结。结果:6例均行手术治疗,全切5例,次全切除1例,术后病理明确诊断均为幕上原始神经外胚叶肿瘤。术后有5例经随访1年显示,术后3个月影像学复发4例,1例未复发,死亡1例,6个月死亡2例,余全部复发;1年死亡4例,1例未死亡。结论:原始神经外胚叶肿瘤在影像学上有一定特征,但特异性不大,最终确诊需依靠病理检查,手术作为首选治疗可一定程度延长患者存活时间,该病预后极差,即便行手术治疗生存期一般不超过1年。
Objective: To investigate the clinical features, surgical treatment and prognosis of intracranial supratentorial primitive neuroectodermal tumors. Methods: This retrospective analysis of the First Affiliated Hospital of Nanjing Medical University from 2000 to 2008 were confirmed by surgery and pathology of supratentorial primitive neuroectodermal tumors in a total of 6 cases, by analyzing the clinical data of each case, the The clinical features of the disease, surgical treatment and prognosis were summarized. Results: All the 6 cases underwent surgical resection. The total resection was performed in 5 cases and the subtotal resection in 1 case. The postoperative pathological diagnosis was performed on the supratentorial primitive neuroectodermal tumor. Five cases were followed up for one year. Four cases were found to have recurred after 3 months of surgery. One case had no recurrence, one case died and two cases died in 6 months. All the cases were relapsed. One case died in 4 cases and one case Did not die. Conclusion: The primitive neuroectodermal tumor has some features in imaging, but its specificity is not so great. The final diagnosis depends on the pathological examination. Surgery as the first choice treatment can prolong the survival time of patients to a certain extent. The prognosis of the disease is extremely poor. Treatment of survival is generally not more than 1 year.