神经母细胞瘤诊治与预后475例的单中心经验  

A single-center review of diagnosis,treatment and prognosis of 475 cases of pediatric neuroblastoma

作　　者：马越 王珊[1] 李长春[1] 周建武[1] 杨超[1] 邓晓斌[1] 赵珍珍[1] 章均[1] 彭亮[1] 孙建 Ma Yue;Wang Shan;Li Changchun;Zhou Jianwu;Yang Chao;Deng Xiaobin;Zhao Zhenzhen;Zhang Jun;Peng Liang;Sun Jian(Department of Surgical Oncology,Children's Hospital of Chongqing Medical University,National Clinical Research Center for Child Health and Disorders,the Ministry of Education Key Laboratory of Child Developmental Diseases Research,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)

机构地区：[1]国家儿童健康与疾病临床医学研究中心,重庆医科大学附属儿童医院肿瘤外科,儿童发育疾病研究教育部重点实验室,儿科学重庆市重点实验室,重庆400014

出　　处：《中华小儿外科杂志》2025年第1期8-14,共7页Chinese Journal of Pediatric Surgery

基　　金：国家重点研发计划"重大慢性非传染性疾病防控研究"重点专项(2018YFC1313000,2018YFC1313004);重庆医科大学附属儿童医院临床医学研究一般项目(NCRCCHD-2019-GP-03)。

摘　　要：目的分析不同危险度分组神经母细胞瘤(neuroblastoma,NB)患儿的预后生存情况,探讨针对高危组(依据美国儿童肿瘤协作组危险度分组)NB患儿采用个体化分层治疗方案的效果,并分析影响高危组NB患儿预后的因素。方法回顾性分析2013年1月1日至2022年7月31日于重庆医科大学附属儿童医院肿瘤外科诊治的475例NB患儿的临床资料,其中男269例,女206例;确诊时中位年龄为29个月,范围在0.1~156.0个月;高危组患儿258例,非高危组患儿217例。所有患儿因经济原因均未接受骨髓移植及抗双唾液酸神经节苷脂抗体治疗,非高危组患儿及2018年1月1日以前收治的高危组患儿治疗方案参照《儿童神经母细胞瘤诊疗专家共识》CCCG-NB-2015方案,在此基础上2018年1月1日以后收治的高危组NB患儿接受个性化的调整治疗方案,重点包括维持期增加节拍化疗及后期中药调理。采用Kaplan-Meier生存曲线对475例患儿行预后生存分析。对258例高危组患儿进行单因素和多因素生存分析,单因素分析采用Log-rank检验,多因素分析采用Cox回归分析。卡方检验比较方案调整前后高危组NB患儿的放疗接受率和肿瘤完全切除率。结果Kaplan-Meier生存曲线显示,非高危组及高危组患儿的5年生存率分别为96.07%和41.82%(P<0.001)。单因素生存分析显示,骨髓转移、骨转移、MYCN基因扩增、血清乳酸脱氢酶水平、是否接受放疗以及是否接受调整方案治疗影响高危组NB患儿的预后(均P<0.05)。多因素生存分析显示是否接受放疗是高危组NB患儿的独立预后影响因素(HR=20.141,95%CI为2.593~156.426,P=0.004)。方案调整前后放疗接受率提高,且差异有统计学意义(18.37%比35.63%,P=0.003);肿瘤完全切除率提高,且差异有统计学意义(74.55%比94.78%,P<0.001);患儿的3年生存率提高(37.82%比65.35%,P<0.001)。结论高危组NB患儿的5年生存率较非高危组NB患儿差。是否接受放疗是高危�Objective To analyze the prognosis and survival of children with neuroblastomas(NB)in different risk groups;to explore the effectiveness of individualized treatment plans for children with high-risk NB;and to analyze the factors influencing the prognosis of children with high-risk NB.MethodsThe clinical data of 475 children who were diagnosed as NB and treated in the Department of Surgical Oncology,Children's Hospital of Chongqing Medical University from January 1,2013 to July 31,2022 were retrospectively analyzed,including 269 males and 206 females with a median age at diagnosis of 29(0.1-156.0)months.According to the risk classification of Children's Oncology Group,there were 258 children in the high-risk group and 217 children in the non-high-risk group.Due to economic reasons,all involved children didn't receive bone marrow transplantation and anti-disialoganglioside GD2 antibody therapy.Children in the non-high-risk group children and those in high-risk group admitted before January 1,2018 were treated based on the CCCG-NB-2015 Regimen.Children with high-risk NB admitted after January 1,2018 received adjusted individualized treatment plans,including increased rhythm chemotherapy in the maintenance period,and traditional Chinese medicine treatment later.Kaplan-Meier survival curve was used to perform prognostic survival analysis on 475 children with NB.Univariate and multivariate survival analyses were conducted on 258 children with high-risk NB,with log-rank test used for univariate analysis and Cox regression analysis used for multivariate analysis.Radiotherapy acceptance rate and tumor complete resection ratio were compared by Chi-square test in high-risk group before and after adjusting the plan.ResultsKaplan-Meier survival analysis showed that the 5-year overall survival(OS)of the non-high-risk group and the high-risk group was 96.07%and 41.82%,respectively(P<0.001).Univariate survival analysis showed that bone marrow metastasis,bone metastasis,MYCN gene amplification,serum lactate dehydrogenase(LDH)leve

关 键 词：神经母细胞瘤 预后 生存率 

分 类 号：R739.4[医药卫生—肿瘤]