单中心低中危神经母细胞瘤患儿的临床特征及长期生存质量  

Clinical characteristics and long-term survival quality in single-center children with low and intermediate-risk neuroblastoma

作　　者：张诗晗 陈诚豪[2] 秦红[3] 蒋持怡 罗茜茜 苏雁[1] 金眉[1] 张大伟[1] 王焕民[3] 曾骐[2] 马晓莉[1] Zhang Shihan;Chen Chenghao;Qin Hong;Jiang Chiyi;Luo Qianqian;Su Yan;Jin Mei;Zhang Dawei;Wang Huanmin;Zeng Qi;Ma Xiaoli(Department of Medical Oncology,Pediatric Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing Key Laboratory of Pediatric Hematology Oncology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China;Department of Thoracic Surgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Surgical Oncology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院儿童肿瘤中心肿瘤内科,北京市儿童血液肿瘤重点实验室,儿科重大疾病研究教育部重点实验室,北京100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院胸外科,北京100045 [3]国家儿童医学中心,首都医科大学附属北京儿童医院肿瘤外科,北京100045

出　　处：《中华实用儿科临床杂志》2021年第21期1623-1627,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：国家科技重大专项(2017ZX09304029);国家自然科学基金培育基金(GPY201703)。

摘　　要：目的总结单中心低中危神经母细胞瘤(NB)患儿的临床特征以及这些患儿生长发育和生存质量的长期随访结果,为进一步的临床研究提供依据。方法回顾性分析2007年3月至2019年6月在首都医科大学附属北京儿童医院诊治的低中危NB患儿的性别、年龄、分期、危险度分组、转移部位等临床特征。Kaplan-Meier方法用于生存分析;WHO Anthro Plus用于计算Z值。结果符合入组标准的低中危NB患儿370例,诊断年龄16.8(1~191)月龄,其中148例(40%)≤12个月。原发瘤灶以纵隔最为多见(177例,47.8%),其次为腹膜位/肾上腺(153例,41.4%)。370例患儿中位随访时间31个月(0.3~157.0个月),5年无事件生存率(EFS)和总生存率(OS)分别为86.2%和96.9%。结束治疗后37例患儿存在生长发育问题,其中生长迟缓22例,低体质量6例,消瘦9例,7.3%(27/370例)的患儿脊柱侧弯。5.5%的患儿心功能轻度异常。5.0%(18/357例)的患儿肾脏损害,其中与原发瘤灶相关12例,手术相关6例。30.2%(95/315例)的患儿化疗后发质发量发生改变,以卷发最为多见。14.9%的患儿性格较治疗前发生改变,以性格急躁最为多见。结论单中心大样本低中危NB患儿5年生存率较高,纵隔是最常见的好发部位,长期生存质量较好,但仍存在治疗相关的不良反应,临床需进一步监测并长期随访。Objective To summarize the clinical characteristics of single-center children with low and intermediate-risk neuroblastoma(NB),report the long-term follow-up results of the growth and survival quality,and provide a basis for further clinical research.Methods Clinical characteristics,including the sex,age,stage,risk of disease,and metastatic site of 370 newly treated children with low and intermediate-risk NB admitted to Hematology Oncology Center,Beijing Children′s Hospital from March 2007 to June 2019 were retrospectively analyzed.Kaplan-Meier method was used for survival analysis.WHO Anthro Plus was used for calculating Z score.Results A total of 370 eligible children with low and intermediate-risk NB were included,with the mean age at onset of 16.8 months(1-191 months).Among them,148 cases(40%)were younger than 12 months old.Mediastinal region was the most common primary site of NB(47.8%,177 cases),followed by retroperitoneum/adrenal gland(41.4%,153 cases).The median follow-up time of 370 patients was 31 months(0.3-157.0 months),the 5-years event free survival(EFS)and 5-year overall survival(OS)were 86.2%and 96.9%,respectively.Thirty-seven cases had growth and deve-lopment problems,of which 22 cases had stunted growth,6 cases had low body mass,9 cases had wasting,and 7.3%(27/370 cases)had scoliosis.5.5%of them had heart damage and 5.0%(18/357 cases)had kidney damage,involving 12 cases related to the primary tumor and 6 cases were surgically related.30.2%(95/315 cases)of them had hair changed after chemotherapy,and curly hair was the most common change.Compared with before treatment,14.9%of the children had a personality change,with an impatient being the most common.Conclusions The 5-year overall survival rate of the single-center large sample of low and intermediate-risk NB was high,mediastinal was the most common primary site of tumor,and the long-term quality of life is good,but there were still treatment-related side effects,and further clinical monitoring and long-term follow-up were needed.

关 键 词：神经母细胞瘤 低中危 生存质量 

分 类 号：R739.4[医药卫生—肿瘤]