神经母细胞瘤CCCG-NB-2015共识多中心应用总结  

Summary of the multicenter application of the CCCG-NB-2015 consensus in neuroblastoma

作　　者：靳燕 袁晓军[2] 赵强 李府[3] 李建新[4] 江莲[5] 卢俊[6] 戴云鹏[7] 赵平 詹江华[8] 李杰 曹嫣娜 李璋琳 杨嘉兴 李忠元 王道威 龚宝成 闫杰 Yan Jin;Xiaojun Yuan;Qiang Zhao;Fu Li;Jianxin Li;Lian Jiang;Jun Lu;Yunpeng Dai;Ping Zhao;Jianghua Zhan;Jie Li;Yanna Cao;Zhanglin Li;Jiaxing Yang;Zhongyuan Li;Daowei Wang;Baocheng Gong;Jie Yan(Department of Pediatric Oncology,Tianjin Medical University Cancer Institute&Hospital,National Clinical Research Center for Cancer,Tianjin Key Laboratory of Cancer Prevention&Therapy,Tianjin's Clinical Research Center for Cancer,Tianjin 300060,China;Department of Hematology and Oncology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Department of Hematology and Oncology,Children's Hospital Affiliated to Shandong University,Jinan 250022,China;Department of Hematology,Wuhan Children's Hospital,Tongji Medical College,Huazhong University of Science&Technology,Wuhan 430015,China;Department of Pediatrics,The Fourth Hospital of Hebei Medical University,Shijiazhuang 050011,China;Department of Hematology,Children's Hospital of Soochow University,Suzhou 215002,China;Department of Pediatrics,Shandong First Medical University Affiliated Provincial Hospital,Jinan 250021,China;Department of Surgery,Tianjin Children's Hospital,Tianjin 300134,China)

机构地区：[1]天津医科大学肿瘤医院儿童肿瘤科,国家恶性肿瘤临床医学研究中心,天津市"肿瘤防治"重点实验室,天津市恶性肿瘤临床医学研究中心,天津市300060 [2]上海交通大学医学院附属新华医院血液肿瘤科 [3]山东大学附属儿童医院(济南市儿童医院)血液肿瘤科 [4]武汉市儿童医院血液科 [5]河北医科大学第四医院儿科 [6]苏州大学附属儿童医院血液科 [7]山东省立医院儿科 [8]天津市儿童医院普外科

出　　处：《中国肿瘤临床》2023年第9期434-442,共9页Chinese Journal of Clinical Oncology

基　　金：国家重点研发计划项目(编号:2018YFC1313000);天津市医学重点学科(专科)建设项目(编号:TJYXZDXK-009A)资助。

摘　　要：目的:对中国多中心应用CCCG-NB-2015共识方案诊治的儿童神经母细胞瘤(neuroblastoma,NB)病例进行回顾性分析总结,以期为改进NB诊疗工作提供有力依据。方法:回顾性分析2013年3月至2020年7月经统一规范的CCCG-NB-2015共识方案诊治的全国多中心500例NB患儿的临床和预后资料,通过Cox单因素和多因素回归分析明确影响NB预后的关键因素;分别绘制神经元特异性烯醇化酶(neuron-specific enolase,NSE)和乳酸脱氢酶(lactate dehydrogenase,LDH)在预测骨髓转移和复发的ROC曲线,明确NSE和LDH的临床价值;比对分析NB两种不同分期系统和危险度分组系统下患儿的预后差异,着重分析中危组人群的队列特征和预后结局。结果:通过单因素分析共确定10个潜在的预后因子,分别为年龄、肿瘤原发部位、INPC分类、骨髓转移、MYCN状态、INSS分期、INRGSS分期、诊断时NSE、LDH水平和影像学定义的危险因素(image-defined risk factors,IDRFs),均P<0.05。Cox多因素分析结果显示与无事件生存率(event-free survival,EFS)密切相关的独立预后因子为INPC分类、骨髓转移、诊断时NSE、LDH水平,与OS密切相关的独立预后因子为NSE、LDH水平及INRGSS分期(均P<0.05);NSE和LDH可以较好地预测NB骨髓转移、复发等事件发生(均P<0.05);INSS分期中非4期和4期的3年EFS分别为93.8%和52.6%;转换为INRGSS分期后,L1、L2、MS和M期的3年EFS分别为94.4%、87.2%、87.5%和53.6%。低危组、中危组和高危组3年和5年的EFS分别为97.1%和97.1%、90.0%和87.3%、53.9%和47.3%;其中INSS分期中3期且伴有INPC为UH型的患儿在CCCG-NB-2015共识方案中被归入高危组,其预后差,5年EFS为65.7%(P<0.05),但却在INRG危险度分组中被列入中危组。结论:NSE和LDH水平在预测NB患儿骨髓是否转移以及预后评价方面具有重要价值。INRGSS分期为预后的独立影响因子,其较INSS分期在对患儿预后评估方面更具有临床意义,推荐使用INRGSS分�Objective:To analyze and summarize the cases of neuroblastoma(NB)in children diagnosed and treated by the multi-center application of CCCG-NB-2015 consensus scheme in China,in order to provide a strong basis for improving the diagnosis and treatment of NB.Methods:The clinical and prognostic data of 500 patients with NB from multiple centers nationwide diagnosed and treated by the unified and standardized CCCG-NB-2015 program were retrospectively analyzed from March 2013 to July 2020,and the key factors affecting the prognosis of NB were identified through Cox univariate and multivariate regression analysis.The ROC curves of NSE and LDH in predicting bone marrow metastasis and recurrence were plotted,and the clinical value of neuron-specific enolase(NSE)and lactate dehydrogenase(LDH)was clarified.The prognostic differences of patients under the two different stages of NB and the risk group system were compared,and the cohort characteristics and prognostic outcomes of the cohort population in the intermediate-risk group were analyzed.Results:A total of 10 potential prognostic factors were identified by univariate analysis,namely age,tumor primary site,INPC classification,bone marrow infiltration,MYCN gene amplification status,INSS stage,INRGSS stage,NSE and LDH levels at diagnosis,and IDRFs(P<0.05).The results of Cox multivariate analysis showed that the independent prognostic factors closely related to event-free survival(EFS)were INPC classification,bone marrow infiltration,NSE and LDH levels at diagnosis,and the independent prognostic factors closely related to OS were NSE,LDH level and INRGSS stage(P<0.05).NSE and LDH can well predict events such as NB bone marrow metastasis and recurrence(P<0.05).The 3-year EFS of INSS 4 and non-INSS 4 was 93.8%and 52.6%respectively.After conversion to INRGSS staging,the 3-year EFS for L1,L2,MS and M were 94.4%,87.2%,87.5%and 53.6%,respectively.The EFS at 3 and 5 years in the low-risk,intermediate-risk and high-risk groups were 97.1%and 97.1%,90.0%and 87.3%,53.9%and 47.3%,resp

关 键 词：神经母细胞瘤 预后 多中心 回顾性分析 

分 类 号：R739.4[医药卫生—肿瘤]