Published online Jul 26, 2021. doi: 10.12998/wjcc.v9.i21.6155
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
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.
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; diag
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.