原始神经外胚层肿1例报道并文献复习.docVIP

原始神经外胚层肿瘤1例报道并文献复习 　　[摘要]中枢神经系统原始神经外胚层肿瘤（PNET）为罕见的恶性肿瘤，术前明确诊断较困难。本文报道1例位于幕下的患者，并汇总2000年至今国内报道的中枢神经系统PNET的文献，统计结果显示其幕上发病占比最高，好发于额叶，男性多于女性，儿童多于成人。该疾病病程短、病死率高，高度恶性，易复发、转移，预后极差。其发病机制尚未完全清楚。对于此疾病需要进一步深入研究，扩大数据量进行分析汇总，对该疾病认识进一步加深，以便于对其病理、病机有更深入地认识，从而提高其诊断、治疗水平，有效提高此类患者的生存质量。 中国论文网 /6/viewhtm　　[关键词]中枢性原始神经外胚层肿瘤；个案报道；文献汇总；恶性肿瘤 　　[中图分类号] R739.4 [文献标识码] A [文章编号] 1674-4721（2018）3（c）-0158-04 　　[Abstract]Primitive neuroectodermal tumor （PNET） in the central nervous system is a rare malignant tumor.It is difficult to make a clear diagnosis pre-operatively.This paper reported 1 subtentorial case，and summarized the PNET in the central nervous system that had been reported in the literature in China since 2000.Statistics showed that the incidence of supratentorial cases accounted for the highest and it often occurred in the frontal lobe，and was more commonly seen in male than female，and in children than adults.The disease had short course with high mortality and malignancy.It was easy to relapse and metastasize，and the prognosis was very poor.The pathogenesis of the disease was not completely clear.This disease need profound study and a large amount of data to make analysis and further deepen the understanding of the disease，in order to have a better understanding of the pathology and pathogenesis，thereby improving the diagnosis and treatment level，and promoting the patients′ quality of life. 　　[Key words]Central primitive neuroectodermal tumor；Case report； Literature summary；Malignant tumor 　　原始神经外胚层肿瘤（primitive neuroectodermal tumor，PNET）是指一组发生于中枢神经系统和周围肌肉、骨骼组织，由原始未分化的小圆细胞构成的恶性肿瘤，它在儿童及青少年中发病率较高，在儿童颅内肿瘤中占2.5%～6%，发生于成人约占0.5%[1]。恶性程度高，易播散，预后极差，确诊需依靠病理。近年来关于PNET的报道逐渐增加，对于PNET认识亦逐渐加深。本院2017年7月收治幕下PNET病例1例，现对其进行报道，并结合目前我国已报道文献进行统计学习，对该病在中枢神经系统中的发病情况进行汇总，对该病影像、病理、诊断等进行初步探讨，以进一步加深广大临床医师对该疾病的认识程度。 　　1临床资料 　　患者女性，57岁，主因头痛头晕10 d入院。查体神清语利，未见明显定位体征。头MRI平扫+增强（图1）示：于右侧小脑半球可见一椭圆形T1WI低T2WI高FLAIR稍高信号影，边界不清晰，周围见片状水肿带环绕，临近脑质，桥脑受压，第四脑室受压变窄，幕上脑室扩张积水，增强扫描病灶边缘明显强化，内部呈部分不均匀强化。术中所见：右侧小脑肿瘤，肿瘤组织为实