目的 探讨儿童横纹肌肉瘤(rhabdomyosarcoma， RMS)的临床特点、综合治疗方法、疗效和预后。方法 回顾性分析武警总医院2003-01至2013-06初治的20例RMS患儿的临床资料，总结儿童RMS的临床特点、综合治疗方法、疗效及预后。结果 20例患儿全部获随访，中位随访时间为38个月;男女比例为2.3∶1;平均发病年龄4.25岁。所有患儿均经病理确诊，依据美国RMS研究组(IRS) 的分期标准，Ⅱ期6例，Ⅲ期9例，Ⅳ期5例;依据世界卫生组织( WHO) 分类，胚胎型17例，腺泡型2例，多形型1例;低危组0例，中危组15例，高危组5例。原发于头颈部18例，四肢1例，睾丸1例。结合上海儿童医学中心RMS 2002方案与美国(IRS)方案制定的方案，7例接受手术+化疗，13例接受以手术、化疗及放疗为主的综合规范治疗，所有患儿治疗后疗效评价:CR12例，PR2例，PD6例，CR率60%。5例高危患儿死亡，其中规范治疗的3例患儿平均随访时间为39.3个月，另2例平均随访时间为14.4个月;13例复发患儿在随访12个月时复发最多，其中7例接受手术联合化疗治疗。结论 胚胎型为儿童RMS多发的病理分型，临床表现缺乏特异性，手术、化疗联合放疗为主的综合规范治疗方法对提高RMS的预后有一定的作用。
Abstract
Objective To investigate the clinical characteristics, the comprehensive treatment, curative effect and prognosis of rhabdomyosarcoma.Methods A retrospective analysis was performed on 20 children with rhabdomyosarcoma who visited China People’s Armed Police Hospital from January 31, 2003 to December 31, 2014. The clinical characteristics, comprehensive treatment efficacy and prognosis of these cases were analyzed.Results All the 20 cases were followed up. The median follow-up time was 38 months; among the 20 cases, male: female 2.3∶1, and the median age was 4.25years old. All the children were with rhabdomyosarcoma confirmed by pathological biopsy. According to The United States Rhabdomyosarcoma Study Group (IRS) staging system, stage I 0 cases stageⅡ 6 cases, stage Ⅲ 9 cases and Ⅳ 5 cases; according to World Health Organization (WHO) classification, 17 cases of embryonic type, two cases of alveolar type and 1 case of polymorphous type; 0 case in low-risk group, 15 cases in the median risk group and 5 cases in high risk group. 18 cases original sites were head and neck, 1 case in limbs and 1 case in testies. Treatment consisted of Shanghai Children’s Medical Center RMS 2002 program combined with the United States (IRS) program development program. Seven cases underwent surgery plus chemotherapy and 13 patients underwent combined standard treatment (surgery, chemotherapy and radiotherapy).The efficacy rates: CR12 cases, PR2 cases, PD6 cases, the CR rate was 60%. 5 cases of high-risk all died, 3 patients’ who underwent standard treatment average follow-up time was 39.3 months, and the other two cases’ average follow-up time was 14.4 months.Almost all 13 cases had relapse at 1 year follow-up, among the 13 cases, seven patients received surgery combined with chemotherapy.Conclusions Embryonal rhabdomyosarcoma is the common pathological type. Clinical manifestations lack specificity.The comprehensive standard treatment method(surgery, chemotherapy, radiotherapy)has a certain role in improving the prognosis of rhabdomyosarcoma.
关键词
横纹肌肉瘤 /
临床特征 /
综合治疗 /
预后
Key words
rhabdomyosarcoma /
clinical features /
comprehensive treatment /
prognostic
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