根治性手术在小儿膀胱前列腺部横纹肌肉瘤中的应用及疗效分析

Role of radical surgery in the management of bladder or prostate rhabdomyosarcoma in children

目的 完整的肿瘤切除是处理小儿膀胱前列腺部横纹肌肉瘤的目标,通过回顾性分析根治性手术治疗小儿膀胱前列腺部横纹肌肉瘤的临床资料,总结临床经验.方法 收集2009年7月至2012年5月间收治的14例小儿原发性膀胱前列腺部横纹肌肉瘤患儿的临床资料.其中,女1例,男13例.共有12例患儿在活检和术前化疗后接受手术.手术时年龄6个月～15岁,平均3.1岁.8例行保留膀胱的肿瘤切除,膀胱尿道吻合术;4例行根治性膀胱切除,原位新膀胱成形术.术后继续化疗,部分患儿接受放疗.术后随访分析手术相关并发症及评估尿控及膀胱功能.结果 14例患儿活检病理报告均为胚胎型横纹肌肉瘤.其中2例在活检后化疗中失访,其余12例均在VAC方案化疗12周后接受手术.IRS分期：3期5例,2期7例.术后随访时间7个月～4.4年,平均25.5个月.随访期间所有患儿均存活.保留膀胱8例术后均无尿失禁,1例术后局部可疑复发,1例出现尿道吻合口狭窄,经后尿道扩张置管术后症状缓解.膀胱切除组4例除1例术后出现白天间歇湿裤,1年后好转外,其余均无尿失禁.3例通过尿道自行排尿,另1例通过清洁间歇导尿排空新膀胱,1例术后出现2次发热性尿路感染,抗感染治疗后痊愈.另1例术后检查发现无症状的双侧膀胱输尿管反流.结论 诱导化疗后的病灶完整手术切除,结合原位新膀胱术是处理小儿膀胱/前列腺部横纹肌肉瘤的一种安全、有效的治疗方法,近期疗效确切.

Objective To analyze the outcomes of radical surgery and orthotopic neobladder reconstruction for bladder or prostate rhabdomyosarcoma （RMS） to evaluate the efficacy of this treatment.Methods From July 2009 to May 2012,14 patients were admitted for primary bladder/prostate rhabdomyosarcoma.There were 13 boys and 1 girl.Twelve of them underwent a second-look operation after pre-chemotherapy biopsy and received a 12-week course of VAC chemotherapy.Their median operative age was 3.08 （0.5-15） years.Four cases underwent radical surgical resection of bladder or prostate and another 8 had local organ-sparing surgery.Bladder function and complications were evaluated clinically.Results All pathological types were embryonal.Two patients were lost to follow-ups after initial biopsy and preoperative chemotherapy.Among 12 surgical patients,the Intergroup Rhabdomyosarcoma Study （IRS） stages were Ⅱ （n =7） and Ⅲ （n =5）.During a median follow up period of 25.5 （7-52.8） months,all patients survived and stayed continent after surgery except for 1 case of diurnal dribbling in radical surgical group.In organ-sparing group,one patient was suspected of local relapse.Another case of urethrostenosis was relieved with urethral sounding.Those with orthotopic neobladder substitution were continent day and night and voided spontaneously or through clearing intermittent catheterization except for 1 case of diurnal dribbling with bladder education.One of them had urinary tract infection twice and was cured after one period of antibiotic therapy.Asymptomatic bilateral vesicoureteral reflux occurred in another case after radical surgery.Conclusions Radical cystoprostatectomy plus orthotopic neobladder substitution is both safe and efficacious for pediatric bladder/prostate rhabdomyosarcoma.