Diagnosis and treatment of bladder embryonal rhabdomyosarcoma in children

doi:10.3877/cma.j.issn.1674-6899.2021.05.007

Abstract

Abstract:

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

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]. Chinese Journal of Laparoscopic Surgery(Electronic Edition), 2021, 14(05): 287-292.

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References 29

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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

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

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

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

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