摘要
目的探讨儿童膀胱胚胎型横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)的临床特点和治疗方法,提高其诊治水平。方法回顾性分析2012年4月至2020年6月解放军总医院第七医学中心附属八一儿童医院儿童泌尿外科收治的13例儿童膀胱ERMS的临床资料,其中男10例、女3例,平均年龄(68.54±41.67)月龄,平均肿瘤最大径(3.84±1.06)cm。术前根据病情决定化学药物治疗、放射治疗或手术治疗,共完成机器人辅助腹腔镜手术8例、腹腔镜手术3例、开放手术2例。其中机器人辅助腹腔镜手术中有膀胱全切并乙状结肠代膀胱并双侧输尿管再植1例、膀胱部分切除并双侧输尿管再植2例、膀胱部分切除并右侧输尿管再植1例、膀胱部分切除并左侧输尿管再植1例、膀胱部分切除并膀胱颈重建1例、膀胱部分切除术2例;腹腔镜膀胱部分切除术3例;开放手术2例,其中膀胱部分切除术并双侧输尿管再植术1例、膀胱部分切除术1例。结果手术均顺利完成,术后病理均为ERMS,13例患儿术后1周开始给予长春新碱+放线菌素D+环磷酰胺(vincristine+actinomycin D+cyclophosphamide,VAC)方案化学药物治疗8个周期。其中1例患儿术后随访第19个月时出现膀胱复发,给予VAC方案化学药物治疗4个周期,彩色多普勒超声复查示肿块较前缩小,行二次手术切除,给予8个周期的VAC方案化学药物治疗,其他患儿术后未见远处转移及复发。结论儿童膀胱ERMS经过手术、化学药物治疗、放射治疗等多学科综合治疗,能取得良好的疗效。
Objective To evaluate the clinical characteristics,diagnosis and treatment of bladder embryonal rhabdomyosarcoma(ERMS).Methods A retrospective study was performed in 13 children with bladder embryonal rhabdomyosarcoma from Apr.2012 to Jun.2020 in Department of Urology,Bayi Children′s Hospital Affiliated to the PLA Army General Hospital.Among these children,10 were male and 3 was female.The average age of these children was(68.54±41.67)months.The mean tumor diameter was(3.84±1.06)cm.According to the condition of the patient,chemotherapy or radiotherapy was performed to achieved the effect of tumor reduction before surgery.The procedures include robotic-assisted laparoscopic radical cystectomy and orthotopic neobladder reconstruction with bilateral ureter reimplantation in 1 patient,robotic-assisted laparoscopic partial cystectomy with bilateral ureter reimplantation in 2 patient,robotic-assisted laparoscopic partial cystectomy with right ureter reimplantation in 1 patient,robotic-assisted laparoscopic partial cystectomy with left ureter reimplantation in 1 patient,robotic-assisted laparoscopic partial cystectomy with bladder neck reconstruction in 1 patient,robotic-assisted laparoscopic partial cystectomy in 2 patients laparoscopic partial cystectomy in 3 patients,partial cystectomy with bilateral ureter reimplantation in 1 patient,partial cystectomy in 1 patient.Results All of 13 operations have been conducted successfully.Postoperative pathology is embryonic rhabdomyosarcoma,all children received VAC chemotherapy for 8 cycles 1 week after operation.Among them,1 patient had bladder recurrence at the 19th month of postoperative follow-up,and was given 4 cycles of VAC chemotherapy.The color Doppler ultrasound showed that the mass was smaller than before.Then the patient received secondary partial cystectomy and VAC chemotherapy for 8 cycles.There was no distant metastasis or recurrence was seen in the remaining children.Conclusions Bladder embryonal rhabdomyosarcoma in children can achieve good results with a m
作者
郭涛
周辉霞
李品
陶天
马立飞
周晓光
曹华林
陶元东
赵扬
朱炜玮
吕雪雪
韩策
卓然
Guo Tao;Zhou Huixia;Li Pin;Tao Tian;Ma Lifei;Zhou Xiaoguang;Cao Hualin;Tao Yuandong;Zhao Yang;Zhu Weiwei;Lyu Xuexue;Han Ce;Zhuo Ran(Medical School of Chinese PLA,Beijing 100853,China;Department of Paediatrics,PLA Army General Hospital,Beijing 100700,China;Department of Urology,Bayi Children′s Hospital Affiliated to the PLA Army General Hospital,Beijing 100700,China;The Second School of Clinical Medicine,Southern Medical University,Guangzhou 510515,China;Guangxi Zhuang Autonomous Region Nanxishan Hospital,Guilin 541120,China)
出处
《中华腔镜外科杂志（电子版）》
2021年第5期287-292,共6页
Chinese Journal of Laparoscopic Surgery(Electronic Edition)
基金
军队计生专项(18JS001)
北京市科技计划(Z18110000718008)。
