原始神经外胚层瘤10年国内文献分析

目的总结我国原始神经外胚层瘤(PNET)的发病情况、治疗及预后,为PNET的诊疗提供资料。方法应用清华同方医学期刊数据库,检索1996年～2006年刊出PNET的文章,对每篇文章进行分析,建立数据库,再进行统计分析。结果共检测到文章44篇,病例149例,发病率有上升趋势,男女之比1.6∶1,中位发病年龄22.7岁,发生于四肢45例(30.2%),躯干104例(69.8%)。135例(90.7%)患者行手术治疗,14例(9.3%)患者未接受手术治疗;明确记载有16例接受了放疗,10例有肿物可评价疗效,4例有效,其中1例完全恢复;74例曾接受化疗,联合化疗中加异环磷酰胺能提高有效率。有明确生存期记录者63例,生存时间40d～8年,其中<12个月45例占(71.4%)。发生于胸部的Askin瘤18例,存活≤6个月9例(56.3%)。结论PNET好发于30岁以下,男性多于女性,发病率有上升趋势,四肢及躯干的骨和软组织都可发生,预后差。早发现、早手术,配合放疗、化疗有希望提高PNET的疗效。

Analysis for 10-year domestic literatures about primitive neuroectodermal tumor

Objective To summarize the incidence,therapy and prognosis of domestic primitive neuroectodermal tumor(PNET)through statistics of recent 10 years reports about PNET,for supplying data to treat malignant tumor more successfully.MethodsTo use the medico-periodical data base of Qinghuatongfang,retrieve the articles of PNET from 1996 to 2006,analyze every article,establish data base and then get the conclusions.Results44 articles and 149 cases were detected.The diseases incidence tended to upgrade.The male patients were 1.6 times more than female patients.The average age of onset was 22.7 years.The location of the tumor was in the extremities in 45 cases(30.2%),104 cases(69.8%)in trunks.135 cases(90.7%)received operation therapy,only 14 cases(9.3%)didn't.16 patients had the records of radiotherapy,the therapeutic effect could be estimated in 10 cases,and 4 cases was effective(including 1 CR).74 patients had the record of chemotherapy.The combination of chemotherapy and iphosphamide could increase the effective rate.63 cases had identified life span,the shortest life time was 40 days and the longest one was 8 years,45 cases(71.4%)survived less than 12 months.18 cases of Askin betide inpectoral,9 cases(56.3%)survived less than 6 months.ConclusionPNET commonly occurs in people below 30 years,male is more likely to suffer from it than female,the incidence is increasing,which can occur in limbs,frames and soft tissues,the prognosis is bad.Early discovery and early operation combined with radiotherapy and chemotherapy will be helpful to enhance the therapeutic effect on PNET.