目的 探讨儿童眼眶横纹肌肉瘤(RMS)的多学科协作诊断及治疗效果.方法 回顾性分析我院眼肿瘤科和儿科2004年11月至2011年12月收治的25例(男15例,女10例,发病年龄3个月至14.3岁,中位年龄7.2岁)原发于眼眶的RMS患儿临床资料及诊疗效果.结果 (1)25例患儿病理分型:胚胎型占80.0%(20/25),腺泡型占20.0%(5/25).(2)按照国际RMS组织分期标准,25例患儿I期3例,II期6例,III期12例,IV期4例.25例患儿分别行不同方法 (手术、化疗、外放疗、125I放射性粒子组织间植入术及自体外周血造血干细胞移植)进行综合治疗.中位随访时间38个月(6～80个月).2年生存率达到88.0%,≥3年估计生存率为74.37%,5年估计生存率为55.7%,死亡5例.结论 儿童眼眶RMS病理分型以胚胎型为主,包括手术、化疗、125I粒子组织间植入及自体外周血干细胞移植等多学科协作模式综合治疗儿童眼眶RMS可以取得良好的临床诊治疗效.%Objective To study the diagnosis and treatment of the strategy of multidisciplinary team treatment on orbital childhood rhabdomysarcoma. Methods The clinical data of 25 children with orbital rhabdomysarcoma was retro -spectively reviewed. Their long-term survival was followed up. From Nov 2004 to Dec 2011 , 25 children with newly diagnosed orbital rhabdomysarcoma (malel5 ,female 10) in Beijing Tongren Hospital were enrolled. Their median age on diagnosis was 7. 2 year old (range 3m ~ 14. 3y). Results (l)The pathology results of 25 cases orbital rhabdomysarcoma showed that embryo type was 80.0% (20/25) and alveolar type was 20. 0% (5/25). (2) Of the 25 cases, 3 were at stage 1,6 at stage Ⅱ, 12 at stage Ⅲ and 4 at stge Ⅳ. They were treated by combined treatment , including surgery , chemotherapy , radiotherapy, I particles local implants and auto peripheral blood stem cell transplantation (APBSCT). The median following-up time of 25 cases rhabdomysarcoma is 38 months until Dec 2011 , 5 cases were dead for cerebral metastasis , total survival rate is 80. 0% (20/25). The 2-year survival rate was 88. 0% ,The estimated 3-year and 5-year survival rate were 74. 37% and 55. 7% respectively. Conclusion The embryo type is the most common pathology type with orbital rhabdomysarcoma in children. Multidisciplinary team treatment is effective model for diagnosis and treatment on childhood orbital rhab -domy sarcoma.
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