Retrospective Study
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 7, 2019; 25(5): 608-621
Published online Feb 7, 2019. doi: 10.3748/wjg.v25.i5.608
Analysis of intrahepatic sarcomatoid cholangiocarcinoma: Experience from 11 cases within 17 years
Dong Kyun Kim, Bo Ra Kim, Jin Sook Jeong, Yang Hyun Baek
Dong Kyun Kim, Yang Hyun Baek, Department of Internal Medicine, Dong-A University College of Medicine, Busan 49201, South Korea
Bo Ra Kim, Department of Radiology, Dong-A University College of Medicine, Busan 49201, South Korea
Jin Sook Jeong, Department of Pathology, Dong-A University College of Medicine, Busan 49201, South Korea
Author contributions: Kim DK is the first author, designed the study, collected and analyzed the data, and drafted the manuscript; Kim BR helped in analyzing the findings of the imaging studies; Jeong JS helped in analyzing the histopathologic findings of the biopsies; Baek YH designed and supervised the study and revised the manuscript for important intellectual content; All authors have read and approved the final version of the manuscript to be published.
Institutional review board statement: This study was reviewed and approved by the Ethics Committee of the Dong-A University Hospital.
Informed consent statement: Patients were not required to provide an informed consent for the study because the analysis used anonymous clinical data that were obtained after the completion of treatment.
Conflict-of-interest statement: All authors declare no conflicts of interest related to this article.
Data sharing statement: No additional data are available.
Open-Access: This 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 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/
Corresponding author: Yang Hyun Baek, MD, PhD, Associate Professor, Department of Internal Medicine, Dong-A University College of Medicine, 26 Daesingongwon-ro, Seo-gu, Busan 49201, South Korea. p100100@dau.ac.kr
Telephone: +82-51-2405678 Fax: +82-51-2401510
Received: October 31, 2018
Peer-review started: October 31, 2018
First decision: December 20, 2018
Revised: January 3, 2019
Accepted: January 14, 2019
Article in press: January 14, 2019
Published online: February 7, 2019
Abstract
BACKGROUND

Intrahepatic sarcomatoid chonalgiocarcinoma (s-CCC) is an extremely rare disease, accounting for less than 1% of hepatobiliary system malignancies, and its pathophysiology is not well known. On the hypothesis that its clinical, serologic, or radiologic diagnosis are not fully understood and its prognosis is poor, we investigated the distinguishing features of s-CCC compared with those of intrahepatic bile duct adenocarcinoma [cholangiocellular carcinoma (CCC)] in patients from a single center.

AIM

To analyze the clinical, serologic, imaging, and histopathologic characteristics of intrahepatic s-CCC patients diagnosed in a single center.

METHODS

The clinical, serologic, imaging, and histopathologic features of 227 patients diagnosed with intrahepatic cholangiocarcinoma (IHCC) in a single medical center during the last 17 years were analyzed. The characteristics of 11 patients with s-CCC were compared with those of 216 patients with CCC.

RESULTS

The number of patients with s-CCC who presented fever and abdominal pain and past history of chronic viral hepatitis or liver cirrhosis (LC) was higher than that of patients with CCC. In imaging studies, patients with s-CCC showed relatively aggressive features. However, no clear distinction was observed between s-CCC and CCC based on other clinical, serologic or radiologic examination results. An accurate diagnosis could be made only via a histopathologic examination through immunohistochemical staining. The clinical course of s-CCC was generally aggressive, and patients had a relatively poor prognosis.

CONCLUSION

In patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.

Keywords: Intrahepatic sarcomatoid cholangiocarcinoma, Immunohistochemical staining, Survival, Prognosis, Surgical resection

Core tip: Intrahepatic sarcomatoid cholangiocarcinoma is rare condition. Patients usually present with an advanced stage of the disease, and they have a poor prognosis. Diagnosis based on histopathologic examination is important because serologic and radiologic examinations cannot help in distinguishing such condition from intrahepatic bile duct adenocarcinoma or other intrahepatic masses. Thus, patients must be diagnosed as early as possible and should receive aggressive treatment, including surgical resection.