Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 7, 2020; 26(25): 3542-3561
Published online Jul 7, 2020. doi: 10.3748/wjg.v26.i25.3542
Practical review for diagnosis and clinical management of perihilar cholangiocarcinoma
Daniele Dondossola, Michele Ghidini, Francesco Grossi, Giorgio Rossi, Diego Foschi
Daniele Dondossola, Giorgio Rossi, General and Liver Transplant Surgery Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan 20122, Italy
Daniele Dondossola, Giorgio Rossi, Department of Pathophysiology and Transplantation, Università degli Studi of Milan, Milan 20122, Italy
Michele Ghidini, Francesco Grossi, Medical Oncology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan 20122, Italy
Diego Foschi, Department of Biomedical and Clinical Sciences "Luigi Sacco", L. Sacco Hospital, Università degli Studi of Milan, Milan 20157, Italy
Author contributions: Dondossola D and Ghidini M drafted the paper; Dondossola D and Foschi D conceptualized the study; Grossi F reviewed the paper; Rossi G and Foschi D approved the final manuscript.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
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:
Corresponding author: Dondossola Daniele, MD, Postdoctoral Fellow, Surgeon, General and Liver Transplant Surgery Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Franscesco Sforza, 35, Milan 20122, Italy.
Received: December 31, 2019
Peer-review started: December 31, 2019
First decision: January 16, 2020
Revised: June 5, 2020
Accepted: June 19, 2020
Article in press: June 19, 2020
Published online: July 7, 2020

Cholangiocarcinoma (CCC) is the most aggressive malignant tumor of the biliary tract. Perihilar CCC (pCCC) is the most common CCC and is burdened by a complicated diagnostic iter and its anatomical location makes surgical approach burden by poor results. Besides its clinical presentation, a multimodal diagnostic approach should be carried on by a tertiary specialized center to avoid miss-diagnosis. Preoperative staging must consider the extent of liver resection to avoid post-surgical hepatic failure. During staging iter, magnetic resonance can obtain satisfactory cholangiographic images, while invasive techniques should be used if bile duct samples are needed. Consistently, to improve diagnostic potential, bile duct drainage is not necessary in jaundice, while it is indicated in refractory cholangitis or when liver hypertrophy is needed. Once resecability criteria are identified, the extent of liver resection is secondary to the longitudinal spread of CCC. While in the past type IV pCCC was not considered resectable, some authors reported good results after their treatment. Conversely, in selected unresectable cases, liver transplantation could be a valuable option. Adjuvant chemotherapy is the standard of care for resected patients, while neoadjuvant approach has growing evidences. If curative resection is not achieved, radiotherapy can be added to chemotherapy. This multistep curative iter must be carried on in specialized centers. Hence, the aim of this review is to highlight the main steps and pitfalls of the diagnostic and therapeutic approach to pCCC with a peculiar attention to type IV pCCC.

Keywords: Perihilar cholangiocarncioma, Liver resection, Biliary drainage, Neo-adjuvant therapy, Type IV cholangiocarcinoma, Klatskin tumor

Core tip: Perihilar cholangiocarncioma (pCCC) is burdened by a complicated diagnostic iter and, due to its anatomical location and biological characteristics, is affected by poor results. Confounding factors, such as biliary decompression, must be avoided during diagnosis and evaluation of longitudinal extension of the tumor. While surgical advances allows the extension of surgical indication (especially for type IV pCCC and portal invasion), adjuvant chemotherapy should be administered to improve post-surgical results. Herein, a highlight on diagnostic and therapeutic management is here provided.