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中华腔镜外科杂志(电子版)

中华腔镜外科杂志(电子版) ›› 2021, Vol. 14 ›› Issue (05) : 287 -292. doi: 10.3877/cma.j.issn.1674-6899.2021.05.007

论著

儿童膀胱胚胎型横纹肌肉瘤13例诊治分析
郭涛1, 周辉霞2,†(), 李品3, 陶天3, 马立飞3, 周晓光3, 曹华林4, 陶元东3, 赵扬1, 朱炜玮1, 吕雪雪1, 韩策1, 卓然2   
  1. 1. 100853　北京，解放军医学院；100700　北京，解放军总医院儿科医学部；100700　北京，解放军总医院第七医学中心附属八一儿童医院泌尿外科
    2. 100853　北京，解放军医学院；100700　北京，解放军总医院儿科医学部；100700　北京，解放军总医院第七医学中心附属八一儿童医院泌尿外科；510515　广州，南方医科大学第二临床医学院
    3. 100700　北京，解放军总医院儿科医学部；100700　北京，解放军总医院第七医学中心附属八一儿童医院泌尿外科
    4. 541120　桂林，广西壮族自治区南溪山医院泌尿外科
  • 收稿日期:2021-08-18 出版日期:2021-10-30
  • 通信作者: 周辉霞
  • 基金资助:
    军队计生专项(18JS001); 北京市科技计划(Z18110000718008)

Diagnosis and treatment of bladder embryonal rhabdomyosarcoma in children

Tao Guo1, Huixia Zhou2,†(), Pin Li3, Tian Tao3, Lifei Ma3, Xiaoguang Zhou3, Hualin Cao4, Yuandong Tao3, Yang Zhao1, Weiwei Zhu1, Xuexue Lyu1, Ce Han1, Ran Zhuo2   

  1. 1. 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
    2. 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
    3. 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
    4. Guangxi Zhuang Autonomous Region Nanxishan Hospital, Guilin 541120, China
  • Received:2021-08-18 Published:2021-10-30
  • Corresponding author: Huixia Zhou
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引用本文：

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. 儿童膀胱胚胎型横纹肌肉瘤13例诊治分析[J/OL]. 中华腔镜外科杂志(电子版), 2021, 14(05): 287-292.

Tao Guo, Huixia Zhou, Pin Li, Tian Tao, Lifei Ma, Xiaoguang Zhou, Hualin Cao, Yuandong Tao, Yang Zhao, Weiwei Zhu, Xuexue Lyu, Ce Han, Ran Zhuo. Diagnosis and treatment of bladder embryonal rhabdomyosarcoma in children[J/OL]. Chinese Journal of Laparoscopic Surgery(Electronic Edition), 2021, 14(05): 287-292.

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。

关键词: 胚胎型横纹肌肉瘤, 膀胱, 儿童, 机器人辅助腹腔镜手术
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 multidisciplinary comprehensive treatment.

Key words: Embryonal rhabdomyosarcoma, Bladder, Child, Robotic-assisted laparoscopic
表1 膀胱胚胎型横纹肌肉瘤患儿的术前情况
序号 性别 年龄(月) 病史 肿瘤最大径(cm) 肿瘤位置 COG术前分期/TNM分期 术前治疗
1 男 27 排尿困难 4.8 弥漫性分布、累及尿道膜部 2期/T2aN0M0 无
2 男 27 间歇无痛肉眼血尿 5.0 膀胱右侧壁 2期/T1aN0M0 无
3 男 95 排尿困难 4.2 膀胱后壁 2期/T1aN0M0 无
4 男 82 排尿困难 4.3 膀胱三角区及尿道膜部 2期/T2aN0M0 VAC×4
5 男 65 排尿困难 5.3 膀胱左侧壁 2期/T1bN0M0 VAC×4
6 男 75 外院盆腔RMS术后复发 2.7 膀胱后壁、三角区及膀胱颈 2期/T2aN0M0 VAC×6, RT×50 Gy
7 男 12 产前发现肾积水，产后超声发现膀胱占位 3.5 膀胱前壁、三角区、双侧输尿管开口、膀胱颈及尿道膜部 2期/T2aN0M0 VAC×4, RT×22 Gy
8 男 46 排尿困难 4.0 膀胱左侧壁 2期/T2aN0M0 VAC×4
9 女 56 排尿困难 3.2 膀胱三角区 2期/T1aN0M0 VAC×4
10 女 86 排尿困难 2.8 膀胱后壁、三角区及双侧输尿管开口 2期/T2aN0M0 VAC×4
11 男 34 排尿困难 2.4 膀胱后壁、左侧输尿管开口 2期/T2aN0M0 VAC×4
12 男 142 排尿困难 5.2 膀胱三角区、双侧输尿管开口、膀胱颈及尿道膜部 3期/T2bN0M0 VAC×4, RT×50 Gy
13 女 144 外院膀胱RMS术后复发 2.5 膀胱三角区、右侧输尿管开口 2期/T2aN0M0 VAC×4
表2 膀胱胚胎型横纹肌肉瘤患儿的术后情况
序号 手术方式 手术时间(min) 术中出血量(ml) 术后住院时间(d) 排尿功能 复发和转移
1 开放膀胱部分切除+双侧输尿管再植术 183 30 18 正常 无
2 开放膀胱部分切除术 130 50 15 正常 无
3 腹腔镜膀胱部分切除术 161 80 21 正常 无
4 腹腔镜膀胱部分切除术 176 50 17 正常 无
5 腹腔镜膀胱部分切除术 156 20 15 正常 有a
6 机器人辅助腹腔镜膀胱部分切除术+膀胱颈重建术 146 30 12 正常 无
7 机器人辅助腹腔镜膀胱根治性切除+乙状结肠原位新膀胱+双侧输尿管再植术 330 30 22 正常 无
8 机器人辅助腹腔镜膀胱部分切除术 139 20 11 正常 无
9 机器人辅助腹腔镜膀胱部分切除术 146 20 12 正常 无
10 机器人辅助腹腔镜膀胱部分切除术+双侧输尿管再植术 198 20 10 正常 无
11 机器人辅助腹腔镜膀胱部分切除术+左侧输尿管再植术 163 30 14 正常 无
12 机器人辅助腹腔镜膀胱部分切除术+双侧输尿管再植术 235 50 10 正常 无
13 机器人辅助腹腔镜膀胱部分切除术+右侧输尿管再植术 178 20 9 正常 无
图1 患儿的MRI示膀胱内可见多发不规则充盈缺损区
1
Skapek SX, Ferrari A, Gupta AA, et al. Rhabdomyosarcoma[J]. Nat Rev Dis Primers, 2019, 5(1): 1.
2
段超，张伟令，孙青，等. 儿童及青少年横纹肌肉瘤多中心临床研究——CCCG-RMS-2016方案近期疗效研究报告[J]. 中国小儿血液与肿瘤杂志， 2020, 25(5): 253-257.
3
中国抗癌协会小儿肿瘤专业委员会，中华医学会儿科学分会血液学组，中华医学会小儿外科学分会肿瘤组. 中国儿童及青少年横纹肌肉瘤诊疗建议(CCCG-RMS-2016)[J]. 中华儿科杂志， 2017，68(10): 724-728.
4
Ognjanovic S, Linabery AM, Charbonneau B, et al. Trends in childhood rhabdomyosarcoma incidence and survival in the united states, 1975-2005[J]. Cancer, 2009, 115(18): 4218-4226.
5
Smith MA, Altekruse SF, Adamson PC, et al. Declining childhood and adolescent cancer mortality[J]. Cancer, 2014, 120(16): 2497-2506.
6
Crist W, Gehan EA, Ragab AH, et al. The third intergroup rhabdomyosarcoma study[J]. Journal of Clinical Oncology, 1995, 13(3): 610-630.
7
Maurer HM, Gehan EA, Beltangady M, et al. The intergroup rhabdomyosarcoma study-Ⅱ[J]. Cancer, 1993, 71(5): 1904-1922.
8
Amer KM, Thomson JE, Congiusta D, et al. Epidemiology, incidence, and survival of Rhabdomyosarcoma subtypes: SEER and ICES database analysis[J]. Journal of Orthopaedic Research, 2019, 37(10): 2226-2230.
9
Winston H, Daniela EB. Rhabdomyosarcoma in adolescent and young adult patients: current perspectives[J]. Adolescent Health Medicine & Therapeutics, 2014, 2014: 115-125.
10
Saltzman AF, Cost NG. Current treatment of pediatric bladder and prostate rhabdomyosarcoma[J]. Current Urology Reports, 2018, 19(1): 11.
11
纪妹. 机器人在子宫内膜癌中的应用[J/CD]. 中华腔镜外科杂志(电子版), 2017, 5(10): 297-299.
12
Agarwal DK, Miest TS, Granberg CF, et al. Pediatric robotic prostatectomy and pelvic lymphadenectomy for embryonal rhabdomyosarcoma[J]. Urology, 2018, 119: 143-145.
13
Li P, Zhou H, Cao H, et al. Robot-assisted laparoscopic radical cystectomy and sigmoid orthotopic neobladder reconstruction for a bladder rhabdomyosarcoma child: case report and literature review[J]. Urology, 2020, 138: 144-147.
14
Anderberg M, Backman T, Annerstedt M. Robot-assisted radical cystoprostatectomy in a small child with rhabdomyosarcoma: a case report[J]. Journal of robotic surgery, 2008, 2(2): 101-103.
15
Cost NG, DaJusta DG, Granberg CF, et al. Robot-assisted laparoscopic retroperitoneal lymph node dissection in an adolescent population[J]. Journal of endourology, 2012, 26(6): 635-640.
16
Arndt C, Rodeberg D, Breitfeld PP, et al. Does bladder preservation (as a surgical principle) lead to retaining bladder function in bladder/prostate rhabdomyosarcoma/results from intergroup rhabdomyosarcoma study iv[J]. Journal of Urology, 2004, 171(6): 2396-403.
17
Heyn R, Newton WA, Raney RB, et al. Preservation of the bladder in patients with rhabdomyosarcoma[J]. Journal of Clinical Oncology, 1997, 15(1): 69-75.
18
Buszek S, Ludmir E, Grosshans D, et al. Patterns of failure and toxicity profile following proton beam therapy for pediatric bladder and prostate rhabdomyosarcoma[J]. Pediatric Blood & Cancer, 2019, 66(11) : 27952.
19
Seitz G, Dantonello TM, Int-Veen C, et al. Treatment efficiency, outcome and surgical treatment problems in patients suffering from localized embryonal bladder/prostate rhabdomyosarcoma: a report from the cooperative soft tissue sarcoma trial cws-96[J]. Pediatric blood & cancer, 2011, 56(5): 718-724.
20
Sardaro A, Carbonara R, Petruzzelli MF, et al. Proton therapy in the most common pediatric non-central nervous system malignancies: an overview of clinical and dosimetric outcomes[J]. Italian journal of pediatrics, 2019, 45(1): 1-13.
21
Leiser D, Calaminus G, Malyapa R, et al. Tumour control and quality of life in children with rhabdomyosarcoma treated with pencil beam scanning proton therapy[J]. Radiotherapy and Oncology, 2016, 120(1) : 163-168.
22
Cotter SE, Herrup DA, Friedmann A, et al.Proton radiotherapy for pediatric bladder/prostate rhabdomyosarcoma: clinical outcomes and dosimetry compared to intensity-modulated radiation therapy[J]. International journal of radiation oncology, biology, physics，2011，81(5): 1367-1373.
23
De Laney TF, Kooy HM. Proton and charged particle radiotherapy[J]. Lippincott Williams & Wilkins, 2008，1: 165-167.
24
Crist WM, Anderson JR, Meza JL, et al. Intergroup rhabdomyosarcoma study-Ⅳ：results for patients with nonmetastatic disease[J]. J Clin Oncol, 2001, 19(12): 3091-3102.
25
Walterhouse D, Pappo A, Meza J, et al. Shorter-duration therapy using vincristine, dactinomycin, and lower-dose cyclophosphamide with or without radiotherapy for patients with newly diagnosed low-risk rhabdomyosarcoma: a report from the soft tissue sarcoma committee of the children′s oncology group[J]. Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2014, 32(31): 3547-3552.
26
Lerner SP, Hayani A, O'Hollaren P, et al. The role of surgery in the management of pediatric pelvic rhabdomyosarcoma[J]. J Urol, 1995, 154(2): 540-545.
27
Seitz G, Fuchs J, Sparber-Sauer M, et al. Improvements in the treatment of patients suffering from bladder-prostate rhabdomyosarcoma: a report from the cws-2002p trial[J]. Annals of Surgical Oncology, 2016, 23(12): 4067-4072.
28
Sparber-Sauer M, Stegmaier S, Vokuhl C, et al. Rhabdomyosarcoma diagnosed in the first year of life: localized, metastatic, and relapsed disease. outcome data from five trials and one registry of the cooperative weichteilsarkom studiengruppe (CWS)[J]. Pediatr Blood Cancer, 2019, 66(6): 27652.
29
Vaarwerk B, Lee JH, Breunis WB, et al. Prognostic relevance of early radiologic response to induction chemotherapy in pediatric rhabdomyosarcoma: a report from the international society of pediatric oncology malignant mesenchymal tumor 95 study[J]. Cancer, 2018, 124(5): 1016-1024.
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