儿童横纹肌肉瘤的临床特点及预后  

Clinical Characteristics and Prognosis of Rhabdomyosarcoma in Children

作　　者：王颖超[1] 宫雪 殷楚云[1] 杜伟闯 李远方 WANG Yingchao;GONG Xue;YIN Chuyun;DU Weichuang;LI Yuanfang(Department of Pediatric Hematology and Oncology,the First Afiliated Hospial of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院儿童血液肿瘤科,河南郑州450052

出　　处：《河南医学研究》2022年第7期1211-1214,共4页Henan Medical Research

摘　　要：目的总结分析儿童横纹肌肉瘤(RMS)的临床特征,比较不同治疗方案对患儿预后的影响。方法收集2013年1月至2019年6月于郑州大学第一附属医院诊断为RMS的47例患儿的临床资料,进行回顾性分析。结果男28例,女19例,发病年龄3-152个月,中位发病年龄为61个月。肿瘤原发部位为头颈部24例(51.1%)、躯干15例(31.9%)、泌尿生殖系统5例(10.6%)、四肢3例(6.4%)。组织学显示胚胎型44例(93.6%)、腺泡型3例(6.4%)。根据危险度分层分为低危组15例、中危组17例、高危组8例和中枢侵犯组7例。有27例手术联合化疗,13例手术联合放化疗,单纯化疗4例,新辅助化疗联合手术3例。共28例患儿应用上海儿童医学中心RS-2009方案,19例患儿应用CCCG-RMS-2016方案。12例死亡,其中2例低危者均为复发后死亡,中危组2例,高危组3例,中枢侵犯组5例。17例复发,失访6例,5例为复发后失访。多因素Cox比例风险回归模型分析显示,危险度分层是影响RMS患儿预后的独立危险因素(P<0.05)。结论头颈部为RMS最常见的原发部位。危险度分层是RMS患儿预后的独立危险因素。上海儿童医学中心RS-2009方案与CCCG-RMS-2016方案对RMS预后影响的差异不大。综合治疗可提高RMS患儿生存率。Objective To summarize and analyze the clinical characteristics of pediatric rhabdomyosarcoma and compare the prognostic effects of different treatment regimens.Methods The clinical data of 47 children diagnosed with rhabdomyosarcoma from January 2013 to June 2019 in the First Affiliated Hospital of Zhengzhou University were collected for retrospective analysis.Results There were 28 boys and 19 girls.The age of onset ranged from3 to 152 months,and the median age of onset was 61 months.The primary tumors were located in head and neck in 24 cases(51.1%),trunk in 15 cases(31.9%),genitourinary system in5 cases(10.6%),and limbs in3 cases(6.4%).The histological findings were 44 cases(93.6%)with embryonal and 3 cases(6.4%)with alveolar type.According to the risk stratification,they were divided into low-risk group of 15 cases,medium-risk groupof 17 cases,high-risk group of 8 cases and central invasion group of 7 cases.There were 27 cases of operation combined with chemotherapy,13 cases of operation combined with radiotherapy and chemotherapy,4 cases of simple chemotherapy and 3 cases of neoadjuvant chemot herapy combined with operation.A total of 28 patients were treated according to the RS-2009 protocol of Shanghai Children's Medical Center,and 19 children were treated according to CCCG-RMS-2016 protocol.Twelve cases died,of which 2 cases in low-risk group died after recurrence,2 cases in medium-risk group,3 cases in high-risk group and 5 cases in central invasion group.There were 17 cases of recurrence,6 cases of loss of follow-up and 5 cases of loss of follow-up after recurrence.Multivariate Cox proportional hazard regression analysis showed that the risk stratification was an independent risk factor for the prognosis of children with RMS.Condusion The head and neck is the most common primary site of RMS.The risk stratification is an independent risk factor for the prognosis of children with RMS.The RS-2009 protocol of Shanghai Children's Medical Center and the CCCG-RMS-2016 protocol have lttle difference in the prog

关 键 词：横纹肌肉瘤 儿童 化疗 

分 类 号：R729[医药卫生—儿科]