儿童外周原始神经外胚层肿瘤/Ewing's肉瘤16例临床分析  

Analysis of 16 cases of peripheralprimitive neuroectodermal tumor in children

作　　者：王莹莹[1] 金仙梅[1] 张超[1] 王立哲 张语桐[1] 常健[1] WANG Yingying;JIN Xianmei;ZHANG Chao;WANG Lizhe;ZHANG Yutong;CHANG Jian(Department of Pediatric Oncology,the First Hospital of Jilin University,Changchun 130021,China)

机构地区：[1]吉林大学第一医院小儿肿瘤科,长春130021

出　　处：《中国小儿血液与肿瘤杂志》2018年第4期194-198,共5页Journal of China Pediatric Blood and Cancer

摘　　要：目的回顾性分析总结儿童外周原始神经外胚层肿瘤/尤文氏肉瘤(pPNET/Ewing's S)的临床特点、疗效及转归。方法收集我科2010年06月-2016年08月诊治的16例pPNET/Ewing'sS患儿的临床资料,并对本组患儿的临床特点、治疗反应、总体生存率进行统计学分析。结果 16例患儿中13例(81%)获得完全缓解,2例(13%)获得部分缓解,1例(6%)进展;患儿总体生存期为11.5～85个月,无事件生存率为63%,1年、3年生存率分别为94%、69%;本组病例中5例复发,其中3例(60%)手术切缘阳性,4例(80%)原发于胸壁或脊椎旁;在5例复发病例中2例病初有远处转移;7例病初伴远处转移患儿中2例复发。复发病例中4例接受二线化疗方案,其中3例仍有缓解。结论儿童pPNET/Ewing's S对放、化疗敏感;手术切缘是重要的预后影响因素;肿瘤原发于胸壁或脊椎旁预后差;远处转移不是影响预后重要因素;复发病例对化疗仍有反应。Objective Retrospectively, to summarize the clinical features, treatment response and prognosis of children with（ Peripheral Primitive Neuroectodermal Tumor/Ewing’s Sarcoma, pPNET/Ewing’s S）. Methods The clinical data of 16 children with pPNET/Ewing’s S from June 2010 to August 2016 were collected, and the clinical characteristics, treatment response and overall survival rate were analyzed statistically. Results Among 16 cases, 13 children（81%） achieved complete response,2 children（13%） achieved partial response, and 1 child（6%） presented progressive disease; The survival time was 11. 5-85 months, the event-free survival rate was 63%, and the 1,3 year overall survival rate was 94%,69%,respectively. In this group, 5 cases relapsed, of which 3 cases（60%） were positive in sectional margin, 4 cases（80%） were originated from the chest wall or the paraspinal regions;Two of the 5 recurrent cases had distant metastases at diagnose, Two of the 7 patients with distant metastases at diagnose relapsed. In the relapsed cases, 4 patients received second-line chemotherapy,and 2 cases of them still got a good response to chemotherapy. Conclusions Children with pPNET/Ewing’s S were sensitive to radiotherapy and chemotherapy, even though recurrent cases also responded to chemotherapy. Positive of sectional margin was an important prognostic factor. The tumors originated from the chest wall or the paraspinal regions had poor prognosis; Distant metastasis at diagnosing was not an important prognostic factor.

关 键 词：儿童 外周型原始神经外胚层肿瘤 治疗 预后 

分 类 号：R739.4[医药卫生—肿瘤]