儿童晚期肝母细胞瘤伴肺转移综合诊疗分析:附12例报告

Treatment of advanced stage childhood hepatoblastoma with pulmonary metastasis : report of 12 cases

目的总结晚期儿童肝母细胞瘤伴肺转移的诊疗经验。方法收集2006年4月—2012年1月收治的12例经病理确诊及分期明确的晚期肝母细胞瘤伴有肺部转移患儿的临床资料,其中男7例、女5例。分析经化疗、介入治疗、手术治疗及自体外周血干细胞移植治疗的临床疗效及随访结果。结果至2012年2月,12例患儿完全缓解3例,部分缓解4例,死亡4例,进展1例,缓解率达到58.3%(7/12),其中10例患儿随访时间超过12个月(12～66月),95%平均生存可信区间是35.3～66.8月,5年生存率估计值为30.41%。结论肺是肝母细胞瘤最常见的转移部位,起病隐匿,预后较差,应注意早期、综合、规律治疗以提高生存率。

Objective To summarize the clinical experience of diagnosis and treatment of advanced stage hepatoblastoma with pulmonary metastasis.Methods Thirty-six cases of hepatoblastoma diagnosed and classified by pathology from April 2006 to January 2012 were enrolled.Twelve of them were with pulmonary metastasis,7 male and 5 female.The clinical effect and follow up of chemotherapy,interventional therapy,operation and autologous peripheral blood stem cell transplantation were analyzed.Results Through February 2012,3 of 12 children achieved complete remission,4 partial remission,4 deceased and 1 deteriorated.The remission rate reached to 58.3%（7 / 12）.The follow up period was over 12 months（12-66 months）,the 95% average survival confidence interval was 35.3-66.8 months and 5-year survival rate was 30.41%.Conclusions Lung is the most common metastatic site of hepatoblastoma.The prognosis of hepatobalstoma with pulmonary metastasis is poor and the onset is insidious.Therefore,it should be treated with early diagnosis and integrated regular treatment to increase survival rate.