Review
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Mar 28, 2015; 21(12): 3472-3479
Published online Mar 28, 2015. doi: 10.3748/wjg.v21.i12.3472
Lessons from rare tumors: Hepatic lymphoepithelioma-like carcinomas
Antonio Solinas, Diego F Calvisi
Antonio Solinas, Department of Biomedical Sciences, University of Sassari, 07100 Sassari, Italy
Diego F Calvisi, Department of Clinical and Experimental Medicine, University of Sassari, 07100 Sassari, Italy
Diego F Calvisi, Institute of Pathology, University of Greifswald, 17489 Greifswald, Germany
Author contributions: Both authors contributed equally to the design of the study, analysis of the data, and drafting of the manuscript.
Conflict-of-interest: The Authors have no conflict of interests to declare.
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: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Antonio Solinas, MD, Professor, Department of Biomedical Sciences, University of Sassari, Viale San Pietro 8, 07100 Sassari. Italy. soltoc@uniss.it
Telephone: +39-79-228443 Fax: +39-79-216282
Received: October 14, 2014
Peer-review started: October 15, 2014
First decision: November 14, 2014
Revised: December 11, 2014
Accepted: February 5, 2015
Article in press: February 5, 2015
Published online: March 28, 2015
Abstract

In this review we focus on lymphoepithelioma-like hepatocellular carcinomas (LEL-HCC) and lymphoepithelioma-like cholangiocarcinomas (LEL-ICC). Despite their rarity, these tumors are of general interest because of their epidemiological and clinical features, and because they represent a distinct model of interaction between the immune system and neoplastic cells. Approximately half of LEL-HCC arise in the context of chronic hepatitis C virus (HCV) infection and have been described both in Eastern and Western patients. By contrast, LEL-ICC is associated in almost all cases with Epstein-Barr virus (EBV) infection and exhibits the same epidemiological features of EBV related malignancies. Compared with classical hepatocellular carcinoma and intrahepatic cholangiocarcinoma of corresponding stage, both LEL-HCC and LEL-ICC are characterized by lower rates of recurrence after surgery and better overall survival. How this behavior is related to distinct genetic alterations and tumor microenvironment is unclear. The pathophysiological mechanisms of lymphoid infiltrations seem to be different among the two groups of tumors. In fact, LEL-HCC frequently arises in the context of inflammatory changes driven by HCV infection, and has been recognized as a variant of classical hepatocellular carcinoma. At variance, lymphocyte recruitment of LEL-ICC is similar to that described in nasopharyngeal carcinoma and gastric LEL, and possibly depends on the expression pattern of latent EBV infection.

Keywords: Lymphoepithelioma, Lymphoepithelioma-like carcinoma, Nasopharyngeal carcinoma, Lymphoepithelioma-like hepatocellular carcinoma, Lymphoepithelioma-like intrahepatic cholangiocarcinoma, Epstein-Barr virus infection

Core tip: Despite their rarity, lymphoepithelioma-like hepatic carcinomas are of general interest because of their peculiar epidemiological and clinical features, and because they represent a distinct model of interaction between the immune system and neoplastic cells. Compared with classical hepatocellular carcinoma and intrahepatic cholangiocarcinoma of corresponding stage, lymphoepithelioma-like hepatic carcinomas are characterized by lower rates of recurrence after surgery and better overall survival. Whether these differences are related to distinct genetic alterations or to the tumor microenvironment is unclear. Here we review the features of these tumors and the mechanisms of lymphoid infiltration.