摘要
目的探讨小儿肝母细胞瘤的临床特点和生存状况,明确其预后影响因素。方法 2009年1月-2013年6月上海市儿童医院初诊并接受治疗的肝母细胞瘤共24例,采用国际儿童肿瘤协会的PRETEXT分期法进行疾病分期,采用手术和化疗相结合的治疗方法,评估其生存率与疾病分期、治疗策略的相关性。结果 24例患者中男15例,女9例,平均年龄(19±12.4)个月。随访至2014年6月30日,平均随访时间(25.6±19.5)个月。Ⅱ期3例(12.5%),Ⅲ期12例(50%),Ⅳ期9例(37.5%),5年总体生存率(OS)和无病生存率(EFS)分别为65.63%±9.83%和43.93%±12.04%。Ⅱ期、Ⅲ期、Ⅳ期的5年OS分别为:100%、91.67%±8.33%和22.22%±14.7%(P=0.001);5年EFS分别为100%、48.89%±13.06%和12.5%±16.67%(P=0.0003)。治疗策略上先行肿瘤根治术3例,均痊愈;余21例行肿瘤穿刺/活检,化疗,手术,再化疗,至今生存13例,复发死亡4例,放弃/失访4例。结论小儿肝母细胞瘤发病年龄小,治疗倾向于手术完整切除为主,辅以术前和术后化疗。疾病分期和肿瘤的完整切除是影响肝母细胞瘤的危险因素。
Objective To investigate the clinical characteristics and prognostic factors of childhood hepatoblastoma. Methods Between January 2009 through June 2013, 24 cases of hepatoblastoma diagnosed at Shanghai children＇s hospital were included in this study. Abide by PRETEXT staging before operation and pathologic diagnosis. Surgery and chemotherapy were applied based on different stage. Evaluation of correlation among the disease stage, treatment and prognosis was done. Results There were 15 boys and 9 girls in these 24 patients, with median age of 19 ± 12.37 months. Till June 30, 2014, the median follow-up time was 25.58 ±19.46 months. According to PRETEXT staging, the number of cases in stage Ⅱ, Ⅲ and Ⅳ was 3 （12. 5%）, 12 （50%） and 9（37.5% ） , respectively. The 5-year overall survival （OS） and event-free survival （EFS） were 65.63%± 9.83% and 43.93% ±12.04% . The 5-year OS for stageⅡ, Ⅲ and Ⅳ were 100% , 91.67% ± 8.33%, 22.22% ± 14.7% （ P = 0. 001 ） respectively, and the 5-year EFS were 100%, 48.89% ±13.06% , 12. 5% ±16. 67% （ P = 0. 0003 ） respectively. Three cases receiving complete tumor excision at primary diagnosis were cured. Others receiving chemotherapies after tumor biopsy, then tumor resection got tumor shrinkage. 13 cases got survival while 4 relapsed and 4 gave up/lost follow up. Conclusions Hepatoblastoma develops in very young age of children. Surgical resection combined with preoperative and postoperative chemotherapy was the main therapy to this disease. Stage and complete resection may be the risk factors for children hepatoblastoma.
出处
《中国小儿血液与肿瘤杂志》
CAS
2015年第2期79-82,共4页
Journal of China Pediatric Blood and Cancer
关键词
肝母细胞瘤
临床特点
预后因素
PRETEXT分期
治疗策略
Hepatoblastoma
Clinical manifestations
Prognostic factors
PRETEXT staging system
Treatment strategies
