小儿睾丸旁横纹肌肉瘤诊治经验分析  

Experience of diagnosing and treating paratesticular rhabdomyosarcoma in children

作　　者：梁海燕[1] 何雨竹 张潍平[1] 杨艳芳 孙宁[1] 宋宏程[1] 韩文文[1] Liang Haiyan;He Yuzhu;Zhang Weiping;Yang Yanfang;Sun Ning;Song Hongcheng;Han Wenwen(Department of Urology,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China;Department of Urology,Henan Children's Hospital,Zhengzhou 450003,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泌尿外科,100045 [2]河南省儿童医院,郑州儿童医院泌尿外科,450003

出　　处：《中华小儿外科杂志》2021年第12期1112-1117,共6页Chinese Journal of Pediatric Surgery

基　　金：北京市医院管理局"登峰"人才培养计划(DFL0151102);北京市医院管理局"扬帆计划":小儿泌尿外科(ZYLX201709);北京市医管局儿科协同发展专项子课题(XTZD20180303)。

摘　　要：目的总结小儿睾丸旁横纹肌肉瘤(rhabdomyosarcoma,RMS)的特点及诊治经验,提高对儿童睾丸旁横纹肌肉瘤认识。方法回顾性总结2008年1月至2018年12月因睾丸旁横纹肌肉瘤就诊于首都医科大学附属北京儿童医院以及河南省儿童医院的21例患儿临床资料。患儿肿瘤位于左侧13例(61.9%),右侧8例(38.1%);根据患儿既往有无手术治疗史分为A(既往无手术史,12例)、B(既往有其他医疗机构手术史,9例)两组。A组患儿年龄为(77.19±56.38)个月,B组年龄为(99.4±17.4)个月。A组患儿肿瘤位于左侧7例、右侧5例;B组位于左侧5例,右侧4例。分析两组病例特点,病理类型,诊治方法及治疗效果。计量资料以Mean±SD表示,采用秩和检验。计数资料的比较采用卡方检验,Kaplan-Meier法进行生存曲线估计,LogRank检验比较两组间生存曲线差异。结果A组12例患儿均采用腹股沟高位入路治疗,B组9例患儿初始手术入路为阴囊入路,再次手术采用腹股沟高位入路扩大切除术。A组随访时间为(99.33±7.34)个月,范围在14~122个月;B组随访时间为(67.75±11.81)个月,范围在6~87个月。A组总体存活率为90.9%(10/11),B组总体存活率为75.0%(6/8),组间比较差异无统计学意义(P=0.28)。A组无进展生存率为90.9%(10/11),B组无进展生存率为50.0%(4/8),组间比较差异有统计学意义(P=0.028)。A组12例患儿的年龄进行了分析,其中7例患儿小于10岁,5例患儿大于10岁。年龄≥10岁患儿的发现肿物至来院就诊的时间为(54.2±43.8)d,年龄<10岁患儿的发现肿物至来院就诊的时间为(21.5±18.9)d,组间比较差异有统计学意义(P=0.018)。年龄≥10岁患儿肿瘤最大径为(5.59±4.37)cm,年龄<10岁患儿肿瘤最大径为(3.1±2.7)cm,组间比较差异有统计学意义(P=0.026)。结论彩色多普勒超声检查对早期发现睾丸旁肿瘤有重要价值,术前准确诊断并行经腹股沟入路高位睾丸根治术可有效提高患儿无进展生存率�Objective To summarize the characteristics and experience of diagnosing and treating pediatric paratesticle rhabdomyosarcoma(RMS),to enhance the understanding of pediatric paratesticle rhabdomyosarcoma and to emphasize the importance of accurate preoperative recognition of the disease and correct surgical approaches.Methods From January 2008 to December 2018,clinical data were retrospectively reviewed for 21 RMS children at both hospitals.The involved side was left(n=13,61.9%)and right(n=8,38.1%).According to whether or not there was a previous surgical history,they were divided into two groups of A(no previous surgical history,n=12)and B(previous surgical history at other medical institutions,n=9).The mean ages of groups A and B were(77.19±56.38)and(99.4±17.4)months respectively.In group A,the involved side was left(n=7)and right(n=5);In group B,the involved side was left(n=5)and right(n=4).Clinical characteristics,pathological types,diagnosis and treatment methods and therapeutic outcomes of two groups were analyzed.SPSS statistical software was utilized for data processing.The measurement data were expressed as mean±standard deviation.Chi-square test was employed for counting data,Kaplan-Meier curve for survival analysis and Log-rank test for comparing survival difference between two groups.Results Group A underwent a high inguinal approach.Nine cases in group B were initially operated via a scrotal approach and followed by extended resection via a high inguinal approach.The mean follow-up periods of groups A and B were(99.33±7.34)(14-122)and(67.75±11.81)(6-87)months respectively.The overall survival rate was 90.9%(10/11)in group A and 75.0%(6/8)in group B without significant inter-group statistical difference(P=0.28).The progression-free survival rate was 90.9%(10/11)in group A and 50%(4/8)in group B with significant inter-group statistical significance(P=0.028).Conclusions Preoperative diagnosis and selecting correct surgical approaches for pediatric RMS are still challenging for pediatric urologists.As

关 键 词：横纹肌肉瘤 横纹肌肉瘤 胚胎型 根治性睾丸切除术 

分 类 号：R737.21[医药卫生—肿瘤]