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World J Clin Oncol. May 24, 2021; 12(5): 335-341
Published online May 24, 2021. doi: 10.5306/wjco.v12.i5.335
Hepatic Langerhans cell histiocytosis: A review
Zhiyan Fu, Hua Li, Mustafa Erdem Arslan, Peter F Ells, Hwajeong Lee
Zhiyan Fu, Hua Li, Mustafa Erdem Arslan, Hwajeong Lee, Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY 12208, United States
Peter F Ells, Department of Gastroenterology, Albany Medical Center, Albany, NY 12208, United States
Author contributions: All authors have contributed to the manuscript and agree with the final version of the manuscript; Fu Z and Lee H are credited with significant contribution to the drafting of the work, literature review of all the sections discussed, the revision of critically important intellectual content, final approval of the published version, and agreement of accountability for all aspects of the work; Li H and Arslan ME are credited with literature review of all sections, and agreement of accountability for all parts of the work; Ells PF is credited with assisting in final content language polishing, and agreement of accountability for all aspects of the work.
Conflict-of-interest statement: The authors have nothing to disclose.
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: Hwajeong Lee, MD, Associate Professor, Department of Pathology and Laboratory Medicine, Albany Medical Center, 47 New Scotland Ave., MC81, Albany, NY 12208, United States. leeh5@amc.edu
Received: February 2, 2021
Peer-review started: February 2, 2021
First decision: March 8, 2021
Revised: March 19, 2021
Accepted: April 25, 2021
Article in press: April 25, 2021
Published online: May 24, 2021
Processing time: 109 Days and 7.7 Hours
Abstract

Hepatic Langerhans cell histiocytosis (LCH) is characterized by proliferation and accumulation of Langerhans cells in the liver, causing liver dysfunction or forming a mass lesion. The liver can be involved in isolation, or be affected along with other organs. A common clinical hepatic presentation is cholestasis with pruritis, fatigue and direct hyperbilirubinemia. In late stages, there may be hypoalbuminemia. Liver biopsy may be required for the diagnosis of hepatic LCH. Histologic finding may be diverse, including lobular Langerhans cell infiltrate with mixed inflammatory background, primary biliary cholangitis-like pattern, sclerosing cholangitis-like pattern, and even cirrhosis at later stages. Because of its non-specific injury patterns with broad differential diagnosis, establishing a diagnosis of hepatic LCH can be challenging. Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation. A definitive diagnosis relies on positive staining with CD1a and S100 antigen. Liver involvement is a high risk feature in LCH. The overall prognosis of hepatic LCH is poor. Treating at an early stage may improve the outcome. Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered. New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy. However, further studies are needed to improve outcome.

Keywords: Langerhans cell; Liver; Cholangitis; CD1a

Core Tip: Hepatic Langerhans cell histiocytosis (LCH) is characterized by proliferation and accumulation of Langerhans cells in the liver, causing liver dysfunction or forming a mass lesion. There are diverse injury patterns on liver biopsy, including lobular Langerhans cell infiltrate with mixed inflammatory background, primary biliary cholangitis-like pattern, sclerosing cholangitis-like pattern, and even cirrhosis at later stage. The overall prognosis of hepatic LCH is poor. New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy.