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Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 21, 2020; 26(19): 2294-2304
Published online May 21, 2020. doi: 10.3748/wjg.v26.i19.2294
Hepatocellular adenoma in the paediatric population: Molecular classification and clinical associations
Elan Hahn, Juan Putra
Elan Hahn, Juan Putra, Division of Pathology, Department of Paediatric Laboratory Medicine, the Hospital for Sick Children, Toronto M5G 1X8, ON, Canada
Elan Hahn, Juan Putra, Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto M5S 1A8, ON, Canada
Author contributions: Hahn E reviewed the literature and drafted the manuscript; Putra J reviewed the literature, acquired the histological images, and edited the final version of the manuscript; all authors approved the final version to be published.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Juan Putra, MD, Assistant Professor, Staff Physician, Division of Pathology, Department of Paediatric Laboratory Medicine, Hospital for Sick Children, 555 University Ave Room 3119, Toronto M5G 1X8, ON, Canada. juan.putra@sickkids.ca
Received: December 30, 2019
Peer-review started: December 30, 2019
First decision: February 19, 2020
Revised: March 29, 2020
Accepted: May 1, 2020
Article in press: May 1, 2020
Published online: May 21, 2020
Processing time: 142 Days and 20.8 Hours
Abstract

Hepatocellular adenomas (HCAs) represent rare, benign liver tumours occurring predominantly in females taking oral contraceptives. In children, HCAs comprise less than 5% of hepatic tumours and demonstrate association with various conditions. The contemporary classification of HCAs, based on their distinctive genotypes and clinical phenotypes, includes hepatocyte nuclear factor 1 homeobox alpha-inactivated HCAs, beta-catenin-mutated HCAs, inflammatory HCAs, combined beta-catenin-mutated and inflammatory HCAs, sonic hedgehog-activated HCAs, and unclassified HCAs. In children, there is a lack of literature on the characteristics and distribution of HCA subtypes. In this review, we summarized different HCA subtypes and the clinicopathologic spectrum of HCAs in the paediatric population.

Keywords: Paediatric; Hepatocellular adenoma; Malignant transformation; Beta-catenin; HNF1A; Glycogen storage disorders

Core tip: Hepatocellular adenomas (HCAs) are uncommon liver tumours with 2 major complications: bleeding and malignant transformation; these lesions are classified based on their distinctive genotypes and clinical phenotypes. HCAs in children may be identified in the setting of conditions such as glycogen storage disorder and familial adenomatous polyposis. However, the molecular subtypes do not always correlate with predisposing risk factors and syndromes. Herein, we will discuss the different subtypes of HCA and the clinicopathological characteristics in children.