放疗及二次根治手术对儿童横纹肌肉瘤的疗效影响分析  

Effect of radiotherapy and second radicalsurgery on rhabdomyosarcoma in children

作　　者：彭晓敏[1] 黎阳[1] 熊稀霖[1] 冯楚础 翁文骏[1] 陈丽容[1] 黄科[1] 周敦华[1] 方建培[1] PENG Xiaomin;LI Yang;XIONG Xilin;FENG Chuchu;WENG Wenjun;CHEN Lirong;HUANG Ke;ZHOU Dunhua;FANG Jianpei(Pediatric Hematology/Oncology,Sun Yat-sen Memorial Hospital,Sun Yat-sen Universitiy,Guangzhou 510120,China)

机构地区：[1]广州中山大学孙逸仙纪念医院儿童肿瘤专科,广东510120

出　　处：《中国小儿血液与肿瘤杂志》2021年第1期29-34,共6页Journal of China Pediatric Blood and Cancer

摘　　要：目的探讨儿童横纹肌肉瘤(RMS)的治疗手段与疗效、预后的关系。方法回顾性分析中山大学孙逸仙纪念医院儿科2013年1月—2017年12月收治的24例儿童RMS的临床资料,总结儿童RMS的治疗策略、疗效,分析放疗及二次根治手术对儿童RMS预后的影响。结果初治儿童RMS共24例,男18例,女6例,发病中位年龄3.6岁(11个月~11岁),其中原发于颌面部14例,颈部1例,腹盆腔2例,肢体4例,生殖系统2例,肝脏1例;病理类型包括胚胎型22例,腺泡型2例;低危组3例,中危组18例,高危组3例。所有患儿均按横纹肌肉瘤全国协作组重庆方案(RMS-CQ-2009)进行化疗,并依据实际病情及疗效反应选择性采取根治手术及放疗。截止2020年6月1日共9例患儿死亡,3年总体生存率(OS)为65.7%。化疗+放疗组的3年OS(80.0%)高于化疗+二次根治术组(50.0%)(χ^(2)=3.896,P=0.048)。对于颌面部RMS,行二次根治术的患儿3年0S(25.0%)低于未行二次根治术的患儿(80.0%)(χ^(2)=6.521,P=0.011);行放疗的患儿3年OS(80.0%)高于未行放疗的患儿(25.0%)(χ^(2)=4.168,P=0.041)。对于非颌面部RMS,行二次根治术和未行二次根治术的患儿3年OS分别是68.6%和66.7%(χ^(2)=0.035,P=0.852),行放疗和未行放疗的患儿3年OS分别为53.3%和80.0%(χ^(2)=0.076,P=0.783)。结论放疗有助于提高儿童RMS生存率,二次根治术并不能显著改善颌面部RMS的预后。Objective To investigate the impact of different treatment strategies on curative effect and prognosis of childhood rhabdomyosarcoma(RMS).Methods A retrospective study based on the clinical notes of the 24 patients with RMS who admitted to Sun Yat-sen Memorial Hospital pediatric department between January 2013 to December 2017 was conducted to summarize the treatment strategies and curative effect and analyze the impact of radiotherapy and secondary radical surgery on the prognosis of RMS in children respectively.Results A total of 24 untreated RMS patients were analyzed,18 males and 6 females,age of onset ranged from 11 months to 11 years old(median:3.6 years).Of the 24 patients,14 were originated in maxillofacial regions,1 in the neck,2 in pelvic cavity,4 in limbs,2 in genitourinary tract and 1 in liver.The histopathologic subtypes included 22 cases of embryonal RMS(ERMS)and 2 cases of alveolar RMS(ARMS).There were 3 cases in low-risk group,18 in intermediate group and 3 in high risk group.All of them received chemotherapy following the Chongqing regimen maded by National Cooperative Group of RMS,and radical surgery and radiotherapy were performed according to the patients′conditions.A total of 9 children died in the follow-up to June 1st,2020.The 3-year overall survival(OS)was 65.7%.The survival rate of patients who received chemotherapy and radiotherapy was higher than those received chemotherapy and secondary radical surgery(3-year OS:80.0%vs 50.0%,χ^(2)=3.896,P=0.048).For maxillofacial RMS,the survival rate of patients who received secondary radical surgery was significantly lower than that of patients who did not(3-year OS:25.0%vs 80.0%,χ^(2)=6.521,P=0.011),the survival rate of patients who received radiotherapy was significantly higher than that of patients who did not(3-year OS:80.0%vs 25.0%,χ^(2)=4.168,P=0.041).In non-maxillofacial region,significant difference of survival rate was shown neither in the patients who received secondary radical surgery and those who did not(3-year OS:68.6%VS 66.7%,χ^

关 键 词：横纹肌肉瘤 儿童 化疗 放疗 

分 类 号：R738.7[医药卫生—肿瘤]