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Cardinale et al. Hepatoma Res 2018;4:20 Hepatoma Research
DOI: 10.20517/2394-5079.2018.46

Review Open Access

Intrahepatic cholangiocarcinoma: review and update

Vincenzo Cardinale *, Maria Consiglia Bragazzi *, Guido Carpino , Sabina Di Matteo , Diletta Overi ,
4
3
2
1
1
Lorenzo Nevi , Eugenio Gaudio , Domenico Alvaro 3
3
4
1 Department of Medico-Surgical Sciences and Biotechnologies, Polo Pontino, Sapienza University of Rome, Rome 00198, Italy.
2 Department of Movement, Human and Health Sciences, Division of Health Sciences, University of Rome “Foro Italico”, Rome
00100, Italy.
3 Department of Medicine and Medical Specialties, Sapienza University of Rome, Rome 00198, Italy.
4 Division of Human Anatomy, Department of Anatomical, Histological, Forensic Medicine and Orthopedics Sciences, Sapienza
University of Rome, Rome 00198, Italy.
*Authors contributed equally.
Correspondence to: Prof. Domenico Alvaro, Department of Medicine and Medical Specialties, Sapienza University of Rome,
Rome 00198, Italy. E-mail: domencio.alvaro@uniroma1.it
How to cite this article: Cardinale V, Bragazzi MC, Carpino G, Di Matteo S, Overi D, Nevi L, Gaudio E, Alvaro D. Intrahepatic
cholangiocarcinoma: review and update. Hepatoma Res 2018;4:20. http://dx.doi.org/10.20517/2394-5079.2018.46
Received: 30 Apr 2018 First Decision: 21 May 2018 Revised: 1 Jun 2018 Accepted: 1 Jun 2018 Published: 19 Jun 2018

Science Editor: Guang-Wen Cao Copy Editor: Jun-Yao Li Production Editor: Cai-Hong Wang

Abstract
Cholangiocarcinoma (CCA) is a heterogeneous group of malignancies that could develop at any level from the biliary
tree. CCA is currently classified into intrahepatic (iCCA), perihilar and distal on the basis of its anatomical location. Of
note, these three CCA subtypes have common features but also important inter-tumor and intra-tumor differences that
can affect the pathogenesis and outcome. A unique feature of iCCA is that it recognizes as origin tissues, the hepatic
parenchyma or large intrahepatic and extrahepatic bile ducts, which are furnished by two distinct stem cell niches, the
canals of Hering and the peribiliary glands, respectively. The complexity of iCCA pathogenesis highlights the need of a
multidisciplinary, translational and systemic approach to this malignancy. This review will focus on the advances of iCCA
epidemiology, histo-morphology, risk factors, molecular pathogenesis, revealing the existence of multiple subsets of iCCA.

Keywords: Cholangiocarcinoma, classifications, inflammation, cells of origin, stem cells, molecular profiling

INTRODUCTION
Cholangiocarcinoma (CCA) is a heterogeneous group of malignancies emerging at any level from the biliary
tree [Figure 1]. CCA is classified into intrahepatic (iCCA), perihilar (pCCA) and distal (dCCA) based on
[1-3]
its anatomical location . Of note, these three CCA subtypes have common features but also important
[1-3]
inter-tumor and intra-tumor differences that can affect the pathogenesis and outcome . The complexity
[4-9]
© The Author(s) 2018. Open Access This article is licensed under a Creative Commons Attribution 4.0
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