5岁以上神经母细胞瘤患者的临床特点及疗效分析  

Clinical features and outcomes of neuroblastoma patients aged above 5 years

作　　者：张谊[1] 张伟令[1] 黄东生[1] 韩涛[1] 支天[1] 李静[1] 易优[1] 文圆[1] 李凡[1] 梅妍妍[1] 杜燕燕[1] ZHANG Yi ZHANG Wei-Ling HUANG Dong-Sheng HAN Tao ZHI Tian LI Jing YI You WEN Yuan LI Fan MEI Yan- Yah DU Yah-Yah.(Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Beijing 100176,China)

机构地区：[1]首都医科大学附属北京同仁医院儿科,北京100176

出　　处：《中国当代儿科杂志》2016年第12期1217-1221,共5页Chinese Journal of Contemporary Pediatrics

基　　金：首都发展基金专项青年项目(首发-2014-4-054);北京市215高层次人才培养项目(BJRC-2016-3-018)

摘　　要：目的总结5岁以上神经母细胞瘤(NB)患儿的临床特点及疗效,为改善其预后提供理论基础。方法回顾性分析54例初治NB患儿临床资料,进行临床特点、疗效总结以及Kaplan-Meier生存分析。结果 54例患儿中男36例、女18例,均为3、4期。以腹膜后占位最多见(76%,41/54),其次为纵隔占位(18%,10/54)、椎管内占位(4%,2/54)和盆腔占位(2%,1/54)。截止至随访日期,54例患儿中存活30例(56%):无病生存23例(77%,其中9例为复发再度化疗后完全缓解)、肿瘤部分缓解6例(20%,均为复发再度化疗患儿)、进展1例(3%,为复发再度化疗后进展);死亡24例(44%),其中22例为复发再度化疗后死亡、2例为初治期间多脏器衰竭死亡;治疗、随访期间共38例复发。Kaplan-Meier生存分析提示:54例患儿平均生存时间53.8个月;3期患儿总生存率(OS)为80%,高于4期患儿(53%),差异具有统计学意义(P<0.01);复发患儿平均生存时间(51.68个月)低于无复发病例(62.57个月),差异有统计学意义(P<0.01)。结论年长儿NB临床分期多属晚期,但规律治疗仍可提高疗效,应增加患儿信心,坚持规范治疗。Objective To investigate the clinical features and outcomes of neuroblastoma（NB） children aged above 5 years,and to provide a theoretical basis for improving prognosis.Methods A retrospective analysis was performed for the clinical data of 54 previously untreated NB children,and their clinical features and outcome were analyzed.The Kaplan-Meier method was used for survival analysis.Results Among the 54 children,there were 36 boys and 18 girls,and all of them had stage 3 or 4 NB.Of all the children,41（41/54,76%） had retroperitoneal spaceoccupying lesions,10（10/54,18%） had mediastinal space-occupying lesions,2（2/54,4%） had intraspinal spaceoccupying lesions,and 1（1/54,2%） had pelvic space-occupying lesions.At the end of the follow-up,30 children（30/54,56%） survived,among whom 23（77%） achieved disease-free survival（9 achieved complete remission after chemotherapy for recurrence）,6（20%） achieved partial remission of tumor（all of them received chemotherapy again due to recurrence）,and 1（3%） experienced progression（with progression after chemotherapy again due to recurrence）; 24 children（44%） died,among whom 22 died after chemotherapy again due to recurrence and 2 died of multiple organ failure during the first treatment.According to the Kaplan-Meier survival analysis,the mean survival time was 53.8 months,and the children with stage 3 NB had a significantly higher overall survival rate than those with stage 4 NB（80% vs 53%; P〈0.01）.The children with recurrence had a significantly lower mean survival time than those without recurrence（51.68 months vs 62.57 months; P〈0.01）.Conclusions Older children often have late-stage NB,but standard treatment can improve their outcomes.

关 键 词：神经母细胞瘤 化疗 手术 预后 儿童 

分 类 号：R739.4[医药卫生—肿瘤]