Nautsch Felix, Ludwig Johannes M, Xing Minzhi, Johnson Kevin M, Kim Hyun S
Department of Radiology and Biomedical Imaging, Yale School of Medicine, Division of Interventional Radiology.
Yale Cancer Center, Yale University, New Haven, CT.
J Clin Gastroenterol. 2018 Mar;52(3):262-267. doi: 10.1097/MCG.0000000000000833.
Primary liver cancer, including Hepatoblastoma (HB) and hepatocellular carcinoma (HCC), in pediatric populations is often fatal. The outcomes are poor despite universal health care access in pediatric patients.
We investigated the sociodemographic factors affecting outcomes in pediatric patients with primary liver cancer.
This is a large population database study of Surveillance, Epidemiology, and End Results cancer registry data from 1973 to 2011. HB and HCC were analyzed regarding age, sex, race, geographic area, and treatment-related information including survival.
In total, 998 patients, the median age at time of diagnosis was 1 year for HB [0-19; 95% confidence interval (CI), 1.5-1.9] and 14 years for HCC (0-19; 95% CI, 12.1-13.3) (P<0.001). Overall Survival (OS) in HB was 374 months (25% failures 19) versus HCC 21 months (25% failures 5; P<0.0001). In HCC, the fibrolamellar subgroup OS was 41 months (32-.) versus 16 months (11-21) in all others [hazard ratio (HR) 2.0; P=0.005]. Diagnosis between 2000 and 2011 (HB: 25% failures not reached; HCC: 38) versus diagnosis 1973 to 1999 (HB: 374; HCC: 12) had different survival (P=0.01; HR 1.9). For HB, OS in patients with age of diagnosis under 2, 25% failures was not reached versus 374 months over the age of 2 (HR 1.7; P<0.0007). African American children with HB had OS of 67 (17-.) versus all others (25% failures 21) and 48% of African American children were diagnosed after the age of 2 versus 34% of whites (HR 1.9; P=0.01).
Later diagnosis and decreased survival in African American children with HB warrants further research.
小儿原发性肝癌，包括肝母细胞瘤（HB）和肝细胞癌（HCC），往往是致命的。尽管儿科患者可获得全民医疗保健，但治疗结果仍很差。
我们调查了影响小儿原发性肝癌患者治疗结果的社会人口学因素。
这是一项基于1973年至2011年监测、流行病学和最终结果癌症登记数据的大型人群数据库研究。对HB和HCC的年龄、性别、种族、地理区域以及包括生存情况在内的治疗相关信息进行了分析。
总共998例患者，HB诊断时的中位年龄为1岁[0 - 19岁；95%置信区间（CI），1.5 - 1.9]，HCC为14岁（0 - 19岁；95%CI，12.1 - 13.3）（P<0.001）。HB的总生存期（OS）为374个月（25%未达到生存目标），而HCC为21个月（25%未达到生存目标）（P<0.0001）。在HCC中，纤维板层样亚型的OS为41个月（32 -...），而其他所有亚型为16个月（11 - 21）[风险比（HR）2.0；P = 0.005]。2000年至2011年诊断的患者（HB：25%未达到生存目标；HCC：38）与1973年至1999年诊断的患者（HB：374；HCC：12）生存情况不同（P = 0.01；HR 1.9）。对于HB，诊断时年龄小于2岁的患者，25%未达到生存目标，而年龄大于2岁的患者为374个月（HR 1.7；P<0.0007）。患HB的非裔美国儿童的OS为67个月（17 -...），而其他所有儿童为（25%未达到生存目标），48%的非裔美国儿童在2岁以后被诊断，而白人儿童为34%（HR 1.9；P = 0.01）。
非裔美国儿童HB诊断较晚且生存期缩短，值得进一步研究。
