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
ARTICLE HIGHLIGHTS
Research background

Intrahepatic sarcomatoid chonalgiocarcinoma (s-CCC) is extremely rare, and its pathophysiology is not well known. Because of the poor prognosis, early diagnosis and aggressive treatment is important.

Research motivation

Clinical, serologic, and imaging findings are known to be not helpful to distinguish s-CCC from intrahepatic bile duct adenocarcinoma (CCC) or other hepatic tumors. There is no established treatment option to prolong survival, except surgery.

Research objectives

This study aimed to analyze the distinct characteristics of s-CCC patients for early diagnosis and appropriate treatment.

Research methods

This retrospective study was conducted in a single center of South Korea for assessment of 11 patients with s-CCC diagnosed for 17 years. We analyzed the clinical, serologic, imaging, and histopathologic features of s-CCC patients and compared with those of CCC patients.

Research results

The patients with s-CCC tended to present abdominal pain or fever as the chief complaint and have past history of liver cirrhosis (LC) or chronic viral hepatitis more frequently, compared with the patients with CCC. In addition, s-CCC showed relatively aggressive features on imaging studies. However, no clear distinction in other clinical and serologic, or radiologic examination results between s-CCC and CCC patients. Only a histopathologic examination with immunohistochemical staining was helpful and essential for an accurate differential diagnosis of s-CCC. The clinical course of s-CCC was relatively aggressive, and patients had poor prognoses. Surgery is generally recommended first, however in this study, we could not obtain meaningful results of the surgical treatment or chemotherapy for s-CCC.

Research conclusions

s-CCC is extremely rare disease which presents aggressive clinical course and poor prognosis. Clinical, serologic, and imaging studies are not helpful in diagnosis of s-CCC. In patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.

Research perspectives

Although s-CCC has a poor prognosis, its pathogenesis and the effects of non-surgical treatment are not well established. In addition, there is no definite strategy for differential diagnosis except a histopathological examination. A large-scale, prospective, and multicenter study involving larger number of patients should be conducted in the future.