Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Sep 21, 2018; 24(35): 3980-3999
Published online Sep 21, 2018. doi: 10.3748/wjg.v24.i35.3980
Pediatric hepatocellular carcinoma
Rajeev Khanna, Sanjeev Kumar Verma
Rajeev Khanna, Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi 110070, India
Sanjeev Kumar Verma, Department of Pediatrics, King George Medical University, Uttar Pradesh 226003, India
Author contributions: Khanna R and Verma SK collectively did literature search and compilation of studies on pediatric hepatocellular carcinoma; the draft was prepared by both Khanna R and Verma SK, and a final editing was done by Khanna R before approval; Khanna R will serve as the guarantor of the manuscript.
Conflict-of-interest statement: There are no potential conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Rajeev Khanna, MD, Associate Professor, Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi 110070, India.
Telephone: +91-9654246963
Received: May 13, 2018
Peer-review started: May 14, 2018
First decision: June 15, 2018
Revised: July 11, 2018
Accepted: August 1, 2018
Article in press: August 1, 2018
Published online: September 21, 2018

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.

Keywords: Hepatocellular carcinoma, Children, Risk-factors, Outcome, Liver transplantation

Core tip: The review elaborately describes various risk factors, treatment options and outcome of children with hepatocellular carcinoma.