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
Abstract

Primary biliary cholangitis (PBC) is a progressive cholestatic liver disease characterized by the presence of highly specific antimitochondrial antibodies, portal inflammation and lymphocyte-dominated destruction of the intrahepatic bile ducts, which leads to cirrhosis. While its pathogenesis remains unclear, PBC that shows histological progression to fibrosis carries a high risk of carcinogenesis; the same is true of viral liver diseases. In patients with PBC, the development of hepatocellular carcinoma (HCC) is rare; the development of combined hepatocellular carcinoma and cholangiocellular carcinoma (cHCC-CCC) is extraordinary. Herein, we report a rare case of PBC metachronously complicated by cHCC-CCC and HCC, which, to the best of our knowledge, has never been reported. We present a case report of a 74-year-old Japanese woman who was diagnosed as PBC in her 40’s by using blood tests and was admitted to our department for further management of an asymptomatic liver mass. She had a tumor of 15 mm in size in segment 8 of the liver and underwent a partial resection of the liver. Subsequent pathological findings resulted in the diagnosis of cHCC-CCC, arising from stage 3 PBC. One year after the initial hepatectomy, a second tumor of 10 mm in diameter was found in segment 5 of the liver; a partial resection of the liver was performed. Subsequent pathological findings led to HCC diagnosis. The component of HCC in the initial tumor displayed a trabecular growth pattern while the second HCC showed a pseudoglandular growth pattern, suggesting that metachronous tumors that arise from PBC are multicentric.

Keywords: Primary biliary cholangitis; Combined hepatocellular carcinoma and cholangiocellular carcinoma; Hepatocellular carcinoma

Core tip: Primary biliary cholangitis (PBC) is a progressive cholestatic liver disease characterized by the presence of highly specific antimitochondrial antibodies, portal inflammation and lymphocyte-dominated destruction of the intrahepatic bile ducts, which leads to cirrhosis. While its pathogenesis remains unclear, PBC that shows histological progression to fibrosis carries a high risk of carcinogenesis; the same is true of viral liver diseases. In patients with PBC, the development of hepatocellular carcinoma is rare; the development of combined hepatocellular carcinoma and cholangiocellular carcinoma (cHCC-CCC) is extraordinary. Herein, we report a rare case of PBC metachronously complicated by cHCC-CCC and HCC, which, to the best of our knowledge, has never been reported.