儿童横纹肌肉瘤8例临床分析

Clinical analysis of rhabdomyosarcoma in 8 children .

目的分析儿童横纹肌肉瘤的临床特点,提高其诊治水平。方法对中国人民解放军总医院1998年1月至2002年10月收治的8例横纹肌肉瘤患儿临床资料进行分析。结果8例患儿中,男5例,女3例;发病年龄7个月至11岁,<5岁者3例;依据美国横纹肌肉瘤研究组(IRS)的分期标准I期2例、Ⅱ期2例、Ⅲ期1例,Ⅳ期3例;原发于头颈部4例、四肢2例,泌尿、消化系统各1例。病理类型胚胎型7例,腺泡型1例。临床表现随原发部位不同有很大差异,基本为肿瘤组织占位、侵蚀后引起。明确诊断依靠病理,治疗以手术、化疗和放疗相结合,放疗总量40～50Gy,化疗共有3种方案VDCA、VAC和VadrC。对Ⅰ、Ⅱ期手术彻底切除肿瘤患儿行VAC、VadrC方案交替共2年,Ⅲ、Ⅳ期患儿前14周用VDCA方案,后VAC、VadrC方案交替共2年。全部患儿2年生存率为37.5%(3/8)。结论儿童横纹肌肉瘤是多发于头颈部,病理多表现为胚胎型的极度恶性软组织肿瘤,早期诊断,合理应用手术、化疗与放疗结合的综合治疗,是提高该肿瘤患儿生存率的关键。

Objective To analyze the clinical characteristics of the childhood rhabdomyosarcoma.Methods Clinical data was analyzed in 8 children with rhabdomyosarcoma.Results From Jan,1998 to Oct,2002,eight children with rhabdomyosarcoma had been diagnosed in our department,5 boys and 3 girls,7 months to 11 years old.Primary site was head and neck (n=4),extremities(n=2),kidney (n=1),bile duct(n=1).2,2,1,3 patients were in stage Ⅰ,Ⅱ,Ⅲ,Ⅳ respectively.Pathological data revealed embryonal rhabdomyosarcoma(n=7) and alveolar rhabdomyosarcoma(n=1).The clinical manifestation was mass in primary site or the tumor erodes.All patients had been diagnosed pathologically.The treatment included multimodal therapy with combination of surgery,chemotherapy,and radiation,whose doses were 40 to 50 Gy.Chemotherapy was given to all patients with VDCA、VAC or VadrC.The patients with pre-Stage II have received chemotherapy with protocol of VAC or VadrC for two years.The protocol of VDCA has been used to the patients with Stage Ⅲand Ⅳ for 14 weeks,then the VAC or VadrC has been used until 2 years later.Two year EFS was 37.5%.Conclusion The head and neck are the most common primary site for rhabdomyosarcoma in children,with mainly histological embryonal rhabdomyosarcoma.Early diagnosis and combination therapy including surgery,chemotherapy,and radiation are key for those with rhabdomyosarcoma to get long EFS.