Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Dec 28, 2017; 9(36): 1378-1384
Published online Dec 28, 2017. doi: 10.4254/wjh.v9.i36.1378
Primary biliary cholangitis metachronously complicated with combined hepatocellular carcinoma-cholangiocellular carcinoma and hepatocellular carcinoma
Ryuta Ide, Akihiko Oshita, Takashi Nishisaka, Hideki Nakahara, Shiomi Aimitsu, Toshiyuki Itamoto
Ryuta Ide, Akihiko Oshita, Hideki Nakahara, Toshiyuki Itamoto, Department of Gastroenterological Surgery, Hiroshima Prefectural Hospital, Hiroshima 734-8530, Japan
Akihiko Oshita, Toshiyuki Itamoto, Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima 734-8551, Japan
Takashi Nishisaka, Department of Pathology Clinical Laboratory, Hiroshima Prefectural Hospital, Hiroshima 734-8530, Japan
Shiomi Aimitsu, Department of Hepatology, Hiroshima General Hospital of West Japan Railway Company, Hiroshima 732-0057, Japan
Author contributions: Ide R and Oshita A made conception and design of this case report; authors other than Ide R and Oshita A, Nishisaka T, Nakahara H, Aimitsu S and Itamoto T contributed to collection and interpretation of data; Ide R and Oshita A wrote the draft manuscript, and other authors performed critical revision of the manuscript; all authors gave final approval of the version to be published; Oshita A has overall responsibility and guarantees the scientific integrity.
Informed consent statement: The patient provided informed consent for the publication of this manuscript and accompanying images.
Conflict-of-interest statement: The authors have no conflict-of-interest to disclose concerning this manuscript.
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: Akihiko Oshita, MD, PhD, Department of Gastroenterological Surgery, Hiroshima Prefectural Hospital, 1-5-54 Ujina-kanda, Minami-ku, Hiroshima 734-8530, Japan. oshita-akihiko@umin.ac.jp
Telephone: +81-82-2541818 Fax: +81-82-2526932
Received: June 17, 2017
Peer-review started: June 19, 2017
First decision: July 20, 2017
Revised: November 8, 2017
Accepted: November 19, 2017
Article in press: November 20, 2017
Published online: December 28, 2017
Processing time: 192 Days and 12 Hours
ARTICLE HIGHLIGHTS
Case characteristics

A 74-year-old Japanese woman was diagnosed as primary biliary cholangitis (PBC) in her 40’s by using blood tests. Imaging studies, including abdominal ultrasonography (US) and computed tomography (CT), and tumor markers consisting of alpha fetoprotein (AFP) and protein induced by vitamin K absence (PIVKA-II) were checked up every 6-12 mo. She was admitted to the authors’ department for further management of an asymptomatic liver mass.

Differential diagnosis

Combined hepatocellular carcinoma and cholangiocellular carcinoma (cHCC-CCC), hepatocellular carcinoma (HCC) and cholangiocellular carcinoma (CCC) were considered from imaging tests.

Laboratory diagnosis

In the initial surgery, serum levels of AFP, PIVKA-II, carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9, and the L3 fraction of AFP were all within normal limits. One year after the initial hepatectomy, tumor marker levels for AFP, PIVKA-II, CEA, and CA 19-9 were within normal limits; only AFP-L3 isoform level was elevated.

Imaging diagnosis

The authors diagnosed both the first and second tumors as HCC from the imaging findings.

Pathological diagnosis

First, hematoxylin-eosin (HE) staining revealed two components, consisting of the trabecular type of HCC and CCC, resulting in the definitive diagnosis of cHCC-CCC. Second, HE staining revealed a pseudoglandular pattern of HCC.

Treatment

The first one was that the tumor was involved in middle hepatic vein (MHV). If radiofrequency ablation was performed, the cooling effect around the MHV would have occurred, leading to the insufficient ablation. The second one was that the tumor was not detected using US preoperatively. Moreover, the tumor was not detected even with intraoperative contrast-enhanced US. Therefore, the authors performed partial resection on the basis of the anatomical structure, including the Glissonean sheath and the hepatic vein.

Related reports

This report relates to this reference: Kobayashi M, Furuta K, Kitamura H, Oguchi K, Arai M, Koike S, Nakazawa K. A case of primary biliary cirrhosis that complicated with combined hepatocellular and cholangiocellular carcinoma. Clin J Gastroenterol 2011; 4: 236-241.

Term explanation

PBC: Primary biliary cholangitis, is marked by slow progressive destruction of the intrahepatic bile ducts, which leads to cirrhosis.

Experiences and lessons

In patients with PBC, it is necessary to check up not only liver function but also carcinogenesis including HCC, CCC and cHCC-CCC.