Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
Department of Pediatrics, King George Medical University, Uttar Pradesh 226003, India.
World J Gastroenterol. 2018 Sep 21;24(35):3980-3999. doi: 10.3748/wjg.v24.i35.3980.
Pediatric hepatocellular carcinoma (HCC) is the second common malignant liver tumor in children after hepatoblastoma. It differs from the adult HCC in the etiological predisposition, biological behavior and lower frequency of cirrhosis. Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille's syndrome and congenital portosystemic shunts are important predisposing factors. Majority of children (87%) are older than 5 years of age. Following mass immunization against hepatitis-B, there has been a drastic fall in the incidence of new cases of pediatric HCC in the Asia-Pacific region. Management is targeted on complete surgical removal either by resection or liver transplantation. There is a trend towards improving survival of children transplanted for HCC beyond Milan criteria. Chemotherapeutic regimens do not offer good results but may be helpful for down-staging of advanced HCC. Surveillance of children with chronic liver diseases with ultrasound and alpha-fetoprotein may be helpful in timely detection, intervention and overall improvement in outcome of HCC.
小儿肝细胞癌（HCC）是儿童期仅次于肝母细胞瘤的第二大常见肝脏恶性肿瘤。它在病因学倾向、生物学行为和肝硬化发生率较低方面与成人 HCC 不同。围生期获得的乙型肝炎病毒、肝肾功能酪氨酸血症、进行性家族性肝内胆汁淤积症、糖原贮积病、Alagille 综合征和先天性门体分流是重要的易患因素。大多数儿童（87%）年龄大于 5 岁。在亚太地区对乙型肝炎进行大规模免疫接种后，小儿 HCC 的新发病例数量急剧下降。治疗的目标是通过切除或肝移植完全切除肿瘤。对于超出米兰标准的 HCC 进行肝移植，患儿的生存率呈上升趋势。化疗方案并不能取得良好的效果，但可能有助于晚期 HCC 的降期。通过超声和甲胎蛋白对慢性肝病患儿进行监测，有助于及时发现、干预 HCC，并整体改善患儿的预后。
