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World J Hepatol. Feb 28, 2017; 9(6): 300-309
Published online Feb 28, 2017. doi: 10.4254/wjh.v9.i6.300
Clinical features, histology, and histogenesis of combined hepatocellular-cholangiocarcinoma
Shweta Gera, Mark Ettel, Gabriel Acosta-Gonzalez, Ruliang Xu
Shweta Gera, Department of Pathology, Montefiore Medical Center, Bronx, NY 10467, United States
Shweta Gera, Mark Ettel, Gabriel Acosta-Gonzalez, Ruliang Xu, Department of Pathology, NYU Langone Medical Center, New York, NY 10016, United States
Author contributions: Gera S contributed to writing introduction, histogenesis and histology section, drafting, designing and revision of the manuscript; Ettel M contributed to writing clinical features, histogenesis, designing and revision of the manuscript; Acosta-Gonzalez G contributed to writing clinical features, imaging and management section and revision of the manuscript; Xu R contributed to supervision, drafting, designing and revision of the manuscript.
Conflict-of-interest statement: All the authors of this study have nothing 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: Ruliang Xu, MD, PhD, Department of Pathology, NYU Langone Medical Center, 560 First Avenue, New York, NY 10016, United States. ruliang.xu@nyumc.org
Telephone: +1-212-2630728 Fax: +1-212-2637916
Received: September 11, 2016
Peer-review started: September 14, 2016
First decision: October 20, 2016
Revised: November 18, 2016
Accepted: January 2, 2017
Article in press: January 3, 2017
Published online: February 28, 2017
Abstract

Combined hepatocellular-cholangiocarcinoma (CHC) is a rare tumor with poor prognosis, with incidence ranging from 1.0%-4.7% of all primary hepatic tumors. This entity will be soon renamed as hepato-cholangiocarcinoma. The known risk factors for hepatocellular carcinoma (HCC) have been implicated for CHC including viral hepatitis and cirrhosis. It is difficult to diagnose this tumor pre-operatively. The predominant histologic component within the tumor largely determines the predominant radiographic features making it a difficult distinction. Heterogeneous and overlapping imaging features of HCC and cholangiocarcinoma should raise the suspicion for CHC and multiple core biopsies (from different areas of tumor) are recommended before administering treatment. Serum tumor markers CA19-9 and alpha-fetoprotein can aid in the diagnosis, but it remains a challenging diagnosis prior to resection. There is sufficient data to support bipotent hepatic progenitor cells as the cell of origin for CHC. The current World Health Organization classification categorizes two main types of CHC based on histo-morphological features: Classical type and CHC with stem cell features. Liver transplant is one of the available treatment modalities with other management options including transarterial chemoembolization, radiofrequency ablation, and percutaneous ethanol injection. We present a review paper on CHC highlighting the risk factors, origin, histological classification and therapeutic modalities.

Keywords: Combined hepatocellular-cholangiocellular carcinoma, Hepatocellular carcinoma, Cholangiocellular carcinoma, Hepatic progenitor cell(s), Histogenesis, Classification

Core tip: Combined hepatocellular-cholangiocarcinoma is a rare tumor with ambiguous data in literature in relation to its clinical features, histogenesis, pathological classification and prognosis. The goal of our study was to review the literature and highlight the new updates on this entity.