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目的探讨幕上原始神经外胚层肿瘤(SPNET)的临床特点、影像学特征和治疗方法及预后。方法回顾性分析10例SPNET患者的临床资料。结果主要临床表现为头痛、呕吐。肿瘤为实性或以实性为主。所有病例均手术治疗,无手术中死亡。术后1例患者次日自动出院。其余9例均于术后辅以化疗或(和)放疗。这9例随访中,1例至今仍存活,存活期42个月;5例于术后4个月～1年内死亡;3例于术后1～3年内死亡。结论幕上原始神经外胚层肿瘤可发生于任何年龄,临床多见于青少年;进展快,主要症状为高颅压;肿瘤呈实性或囊实性,可伴有出血;需采用综合治疗,其中手术全切是主要的治疗方法,术后辅以化疗或(和)放疗;预后差,易复发,平均生存时间短。
Objective To investigate the clinical features, imaging features, treatment and prognosis of supratentorial primitive neuroectodermal tumors (SPNET). Methods The clinical data of 10 SPNET patients were retrospectively analyzed. The main clinical manifestations of headache, vomiting. Tumors are solid or solid. All cases were surgically treated and died without surgery. One patient was discharged automatically the next day after surgery. The remaining 9 cases were supplemented with chemotherapy or (and) radiotherapy. Among the 9 cases, 1 patient still survived 42 months after operation, 5 patients died within 4 months to 1 year after operation, and 3 patients died within 1 to 3 years after operation. Conclusion The supratentorial primitive neuroectodermal tumors can occur at any age, clinical more common in adolescents; rapid progress, the main symptom is high intracranial pressure; solid tumors or cystic solidity, may be associated with bleeding; need to use comprehensive treatment, including surgery Full cut is the main treatment, postoperative chemotherapy or (and) radiotherapy; poor prognosis, easy to relapse, the average survival time is short.