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©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
Complicated course of biliary inflammatory myofibroblastic tumor mimicking hilar cholangiocarcinoma: A case report and literature review
Sandra Strainiene, Kotryna Sedleckaite, Juozas Jarasunas, Ilona Savlan, Juozas Stanaitis, Ieva Stundiene, Tomas Strainys, Valentina Liakina, Jonas Valantinas
Sandra Strainiene, Ilona Savlan, Juozas Stanaitis, Ieva Stundiene, Valentina Liakina, Jonas Valantinas, Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
Kotryna Sedleckaite, Vilnius University, Faculty of Medicine, Vilnius 01513, Lithuania
Juozas Jarasunas, Department of Radiology, Nuclear Medicine and Medical Physics, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
Tomas Strainys, Clinic of Anesthesiology and Intensive Care Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
Valentina Liakina, Department of Chemistry and Bioengineering, Faculty of Fundamental Science, Vilnius Gediminas Technical University, Vilnius 10223, Lithuania
Author contributions: Strainiene S, Sedleckaite K, Jarasunas J, and Strainys T performed the patient’s data extraction; Strainiene S, Sedleckaite K, Jarasunas J, and Strainys T wrote the original manuscript and reviewed the literature; Savlan I was the gastroenterologist who followed-up the patient; Jarasunas J was the radiologist in this case and selected the radiological images; Stanaitis J was the endoscopist who performed most of the procedures; Savlan I, Liakina V, and Stundiene I reviewed and edited the manuscript; Stanaitis J, and Valantinas J revised the manuscript for important intellectual content; All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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:
http://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Sandra Strainiene, MD, Doctor, Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania. sandra.strainiene@santa.lt
Received: March 29, 2021
Peer-review started: March 29, 2021
First decision: April 28, 2021
Revised: April 30, 2021
Accepted: May 20, 2021
Article in press: May 20, 2021
Published online: July 26, 2021
BACKGROUND
The inflammatory myofibroblastic tumor (IMT) is a rare, idiopathic, usually benign, mass-forming disease with myofibroblastic proliferation and a varying amount of inflammatory cells. Although it can affect various organs, the biliary tract is a rare localization of primary IMT, clinically, endoscopically and radiologically imitating cholangiocarcinoma. The treatment options are based only on clinical practice experience.
CASE SUMMARY
A 70-year-old woman was referred to our center due to progressive fatigue, weight loss, abdominal pain, night sweats, and elevated liver enzymes. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed proximal common hepatic duct and hilar biliary strictures extending bilaterally to lobular bile ducts. Although initial clinical, endoscopic and radiological signs were typical for hilar cholangiocarcinoma, histological examination showed no signs of malignancy. In total, 8 biopsies using different approaches were performed (several biopsies from dominant stricture during ERCP and direct cholangioscopy; ultrasound-guided liver biopsy; diagnostic laparoscopy with liver and lymph node biopsies). Histological examination revealed signs of IMT, and the final diagnosis of biliary IMT was stated. Although IMT is usually a benign disease, in our case, it was complicated. All pharmacological treatment measures were ineffective. The patient still needs permanent stenting, suffers from recurrent infections and mechanical jaundice. Despite that, the patient already survived 24 mo.
CONCLUSION
IMT presenting with hilar biliary strictures is a unique diagnostic and clinical challenge as it is indistinguishable from cholangiocarcinoma, and there are no evidence-based treatment options. Our goal is to increase the understanding of this rare disease and its possible course.
Core Tip: Biliary inflammatory myofibroblastic tumor (IMT) is a rare, idiopathic, usually benign, mass-forming disease with myofibroblastic proliferation and the varying amount of inflammatory cells that can occur in almost every organ. IMTs of the biliary ducts are an uncommon cause of obstructive jaundice. The clinical and radiological presentation usually mimics cholangiocarcinoma (Klatskin tumor). However, histological examination shows no malignancy. We present a rare, difficult to diagnose and treat biliary IMT, which was unresponsive to the pharmacological treatment and complicated by recurrent infections and disease progression. The global experience towards diagnosing and treating this disease is limited and based mostly on clinical practice experience.
