儿童泌尿生殖系统横纹肌肉瘤17例

Rhabdomyosarcoma in Genitourinary in 17 Children

目的探讨儿童泌尿生殖系统横纹肌肉瘤(RMS)的诊断和治疗方法。方法回顾性研究1978年6月-2010年3月本院收治的17例泌尿生殖系统RMS患儿的临床资料。患儿均采用以手术为主,联合术前术后化疗、放疗的治疗方法。采用SPSS13.0软件进行统计学处理,运用Kaplan-Meier曲线进行生存率分析。结果本组患儿中肿瘤来源于膀胱10例、前列腺5例、阴道2例。手术行肿瘤根治性切除加膀胱全切5例,肿瘤根治性切除加膀胱部分切除7例,肿瘤姑息性切除1例,活检4例。术后病理诊断胚胎型16例,腺泡型1例。根据美国RMS协作组(IRS)标准进行术后分期,Ⅰ期6例,Ⅱ期5例,Ⅲ期4例,Ⅳ期2例。术前化疗3例,术后化疗9例。术后放疗1例。手术完整切除率70.1%(12/17例),膀胱保存率58.3%(7/12例)。Kaplan-Meier生存率曲线图分析,本组预期存活时间(14.025±3.484)个月,2a生存率37.5%。结论泌尿生殖系统RMS治疗必须兼顾生命和保全器官功能。手术联合术前术后化疗及术后放疗可以提高患者生存率。

Objective To study the methods of diagnosis and management about childhood rhabdomyosarcoma（RMS） in the genitourinary. Methods Seventeen cases with genitourinary RMS in the First Affiliated Hospital of Guangxi Medical University from Jun. 1978 to Mar. 2010 were studied retrospectively. The treatment of all cases included muhimodal therapy with combination of surgery, chemotherapy and radiation. Clinical data was analyzed by SPSS 13.0 statistics software and survival rate was analyzed by Kaplan -Meier curve. Results The tumor origin from bladder were 10 cases,prostate 5 cases,vagina 2 cases. The tumor and bladder radical excision were 5 cases,and the tumor radical excision and bladder partial resection were 7 cases,and the tumor partial resection was 1 case,and the tumor biopsy were 4 cases. The postoperative pathological diagnosis were embryonal 16 cases, alveolar 1 case. According to Intergroup Rhabdomyosarcoma Study（ IRS）, the postoperative staging were stage Ⅰ 6 cases,stage Ⅱ 5 cases,stage Ⅲ 4 cases,stage Ⅳ 2 cases. The preoperative chemotherapy were 3 cases. The postoperative chemotheropy were 9 cases. The postoperative radiotherapy was 1 case. The rate of radical surgery was 70. 1% （ 12/17 cases）, and the rate of bladder＇s preservation was 58.3 % （7/12 cases）. According to Kaplan -Meier survival curve diagram, the expect mean survival time were （ 14. 025 ± 3. 484） months, and the event - free survival at 2 years was 37.5%. Conclusions The strategy, of RMS treatment must be considerated two things,how to resect the tumor and how to preserve the bladder. Combination therapy including surgery,chemotherapy, and radiation can increase the survival rate.