单中心婴儿横纹肌肉瘤的临床特征及预后分析  

Clinical and prognostic analysis of rhabdomyosarcoma in infants:a single-center report

作　　者：徐娜 段超[1] 王生才[2] 金眉[1] 于彤[3] 何乐建 秦红[5] 马晓莉[1] Xu Na;Duan Chao;Wang Shengcai;Jin Mei;Yu Tong;He Lejian;Qin Hong;Ma Xiaoli(Medical Oncology Department,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 Otolaryngology Head and Neck Surgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Radiology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Pathology,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耳鼻咽喉头颈外科,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影像科,北京100045 [4]国家儿童医学中心,首都医科大学附属北京儿童医院病理科,北京100045 [5]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》2022年第4期299-303,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：国家科技重大专项(2017ZX09304029004);北京市医院管理局儿科学科协同发展中心儿科专项(XTYB201803)。

摘　　要：目的总结分析婴儿横纹肌肉瘤(RMS)患儿的临床特征及预后,进一步提高对婴儿RMS的认识。方法选择2006年5月至2019年6月在首都医科大学附属北京儿童医院肿瘤内科诊治的所有年龄<12个月RMS患儿,回顾性分析初诊时患儿年龄、瘤灶部位及大小、病理分型、临床分期分组等临床特征。随访时间截至2019年12月31日。采用Kaplan-Meier生存分析法分析患儿3年无事件生存(EFS)率。结果共入组RMS患儿15例,占同期RMS患儿的4.9%,其中男6例,女9例;中位诊断年龄7.0个月(3.0~11.5个月)。原发瘤灶以头颈部最常见[40.0%(6例)],其次为四肢[26.7%(4例)]和其他部位[26.7%(4例)],泌尿生殖系统为6.7%(1例)。胚胎型RMS占46.6%(7例),腺泡型RMS 26.7%(4例),梭形细胞型RMS 26.7%(4例)。美国横纹肌肉瘤研究组(IRS)-Ⅲ期10例(66.7%),Ⅳ期1例(6.7%)。中危组10例(66.6%),低危组4例(26.7%),高危组1例(6.7%)。2例患儿原发瘤灶>5 cm,3例患儿明确淋巴结受累,1例患儿在诊断时发生肺转移。患儿均进行化疗,13例化疗前手术;1例化疗后手术;1例未手术。仅3例患儿进行放疗:1例患儿行粒子植入;2例行外放疗。15例婴儿RMS患儿中5例出现复发或进展[3年EFS率为(59.1±14.5)%],2例死亡[3年总生存率为(80.8±12.6)%]。结论单中心婴儿期RMS患儿中位诊断年龄为7个月,以头颈部为常见,近半数患儿原发瘤灶部位为预后不良部位,病理亚型中超过1/4为梭形细胞型。局部治疗欠缺可能是导致局部进展或复发的主要因素。Objective To analyze the clinical characteristics and prognosis of infants with rhabdomyosarcoma(RMS),thus enhancing the understanding of this disease.Methods Clinical data of all RMS patients younger than 12 months treated in the Hematology Oncology Center,Beijing Children′s Hospital,Capital Medical University from May 2006 to June 2019 were retrospectively analyzed,including the age,gender,histological type,tumor primary site,tumor size,and the prognosis.Patients were followed up until December 31,2019.The 3-year event free survival(EFS)rate of children was performed by plotting the Kaplan-Meier survival curves.Results A total of 15 RMS children younger than 12 months were enrolled,accounting for 4.9%of all RMS cases in the same period,including 6 males and 9 females.The median age at diagnosis was 7.0 months(3.0-11.5 months).Classified by the primary site,40.0%(6 cases)located in the head and neck,followed by 26.7%(4 cases)located in the limbs,26.7%(4 cases)located in other parts,and 6.7%(1 case)located in the urogenital system.Embryonal RMS,alveolar RMS and spindle cell RMS accounted for 46.6%(7 cases),26.7%(4 cases),and 26.7%(4 cases),respectively.Ten cases(66.7%)were stage Intergroup Rhabdomyosarcoma Study(IRS)-Ⅲand 1 case(6.7%)was in stageⅣ.There were 10 cases(66.6%)in the middle-risk group,4 cases(26.7%)in the low-risk group,and 1 case(6.7%)in the high-risk group.Two cases had a larger than 5 cm primary tumor;lymph node involvement was confirmed in 3 cases,and pulmonary metastasis occurred in 1 case at the time of diagnosis.All children were treated with chemotherapy,and 13 cases received postoperative chemotherapy and 1 case received preoperative chemotherapy.One case were not operated.Only 3 children underwent radiotherapy,including 1 case underwent particle implantation and 2 cases received external radiotherapy.Among the 15 children with RMS,5 cases had relapse and disease progression with the 3-year EFS rate of(59.1±14.5)%,and 2 died with the 3-year overall survival rate of(80.8±12.6)%.Conclu

关 键 词：横纹肌肉瘤 婴儿 治疗 预后 

分 类 号：R738.6[医药卫生—肿瘤]