儿童原始神经外胚叶肿瘤的临床特征(附4例临床报告及文献复习)

Fetures of primitive neuroectodermal tumors in children(4 cases report and rewiew of the literatures)

目的 探讨儿童大脑神经外胚叶肿瘤的临床特点及治疗方法。方法 对我院 1999年 1月至 2 0 0 3年 1月收治的 4例小儿大脑神经外胚叶肿瘤的临床资料进行回顾性分析。结果 4例病人 1例位于额叶 ,2例位于顶叶 ,1例位于颞叶。CT显示为高或等密度影 ,MRI为短T1长T2信号 ,增强后均匀强化 ,肿瘤有明显界线。 4例均在显微镜下全切 ,病理诊断 :原始神经外胚叶肿瘤。术后均行放疗。术后 8个月～ 18个月复发。结论 小儿大脑原始神经外胚叶肿瘤为高度恶性的肿瘤 。

Objective To explore the diagnosis and treatment of primitive neuroectodermal tumors(PNETs)in children.Methods Four children with primitive neuroectodermal tumors were retrospectively reviewed from January 1999 to January 2003.Results The lesions located at front lobe in one patient,2 patients with lesions located at parietal lobe,1 patient with lesion located at temporal lobe.The tumors was hyperintense or isointense on CT scan.On MRI of the head,the tumor was hyperintense on T1-weighted sequence,with increased signal on T2-weighted images,and contrast-enhanced after administration of gadolinium.All cases were totally resected by microsurgical technique,all tumors were PNETs confirmed pathologically and received postoperative irradiation.The tumors relapsed between 8 months and 18 months after the initial surgery.Conclusion PNETs in children are malignant,the patients received operation and postoperative irradiation can improve their survival period.The prognosis of PNETs is bad.