218 例小儿肝细胞癌病例回顾。

A review of 218 pediatric cases of hepatocellular carcinoma.

机构信息

Department of Surgery, Division of Pediatric Surgery, DeWitt-Daughtry Family, University of Miami Miller School of Medicine, Miami, FL 33136, USA.

出版信息

J Pediatr Surg. 2014 Jan;49(1):166-71; discussion 171. doi: 10.1016/j.jpedsurg.2013.09.050. Epub 2013 Oct 5.

DOI:10.1016/j.jpedsurg.2013.09.050

PMID:24439603

Abstract

PURPOSE

This study evaluates the incidence trends and clinical outcomes of children with hepatocellular carcinoma (HCC) and assesses factors predictive of patient survival.

METHODS

The Surveillance, Epidemiology, and End Results registry was queried from 1973 to 2009 for all patients between ages 0 and 19 with primary HCC. Demographics, tumor histology, surgical intervention, and patient survival were collected.

RESULTS

Overall, 218 patients were identified. The annual age-adjusted incidence was 0.05 cases per 100,000 in 2009. Fibrolamellar subtype tumors were exclusive to children >5years old and exhibited greater survival compared to non-fibrolamellar subtype (57% vs. 28%, respectively, p=0.002). Tumor extirpation for patients with resectable disease significantly improved overall survival at 5years compared to no surgery (60% vs. 0%, respectively, p<0.0001). Overall 5-, 10- and 20-year survival for the entire cohort was 24%, 23%, and 8%, respectively. Independent prognostic factors of lower mortality according to multivariate analysis were surgical resection (hazard ratio (HR)=0.18), non-Hispanic ethnicity (HR=0.52), and local disease at presentation (HR=0.46).

CONCLUSION

Over the past four decades, the incidence of HCC has remained relatively stable. Children of Hispanic ethnicity have high mortality rates. However, HCC resection for curative intent significantly improves outcomes.

摘要

目的

本研究评估了儿童肝细胞癌（HCC）的发病趋势和临床结果，并评估了预测患者生存的因素。

方法

从 1973 年至 2009 年，通过监测、流行病学和最终结果登记处，对所有年龄在 0 至 19 岁之间的原发性 HCC 患者进行了检索。收集了人口统计学、肿瘤组织学、手术干预和患者生存情况。

结果

共有 218 名患者被确定。2009 年，年龄调整后的年发病率为每 10 万人中有 0.05 例。纤维板层样肿瘤仅见于年龄>5 岁的儿童，与非纤维板层样肿瘤相比，生存时间更长（分别为 57%和 28%，p=0.002）。对于可切除疾病的患者，肿瘤切除可显著提高 5 年总生存率，与无手术相比（分别为 60%和 0%，p<0.0001）。整个队列的 5 年、10 年和 20 年生存率分别为 24%、23%和 8%。根据多变量分析，降低死亡率的独立预后因素是手术切除（风险比（HR）=0.18）、非西班牙裔种族（HR=0.52）和局部疾病（HR=0.46）。