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Hawkins N, Sun D, Pornkul P, Bae K, Ben David M. Hilar and Extrahepatic Inflammatory Pseudotumour: A Case Report and Systematic Literature Review. Cureus 2025; 17:e79727. [PMID: 40017578 PMCID: PMC11865861 DOI: 10.7759/cureus.79727] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/22/2025] [Indexed: 03/01/2025] Open
Abstract
Inflammatory pseudotumor (IPT) is a benign inflammatory lesion that is exceptionally rare in the biliary tree. Its clinical and radiological presentation mimics neoplastic disease, so diagnosis often relies on histology. Surgical resection is the mainstay of treatment. A case of histologically confirmed IPT in a 52-year-old female, successfully managed with surgical resection, is presented. A systematic literature review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to identify case reports and case series of IPT involving the extrahepatic bile ducts and liver hilum. Searches of PubMed and Embase (from January 1960 to December 2024) yielded 23 original articles that met inclusion criteria. Data on clinical presentation, management, and outcomes were analyzed. A total of 33 cases of hilar and extrahepatic IPT have been reported in the literature. Obstructive jaundice was the most common presentation (79% n=26/32) accompanied by biochemical elevation of liver function tests (91% n=21/23) and bilirubin (77% n=17/22). Surgical resection was the most common treatment (82% n=27/33) with excellent outcomes and only one case of recurrence. However, the follow-up period was relatively short (median of one year). Of five cases initially treated with steroids, three were successfully managed with steroids alone. The other two cases proceeded to surgical resection due to disease progression. Serum immunoglobulin-G4 was high in successfully managed cases and not reported in failed cases. Hilar and extrahepatic IPT is a rare pathology with a similar presentation to neoplastic disease, which can make diagnosis and management challenging. Surgical resection is the mainstay of management, however, in select cases, preoperative biopsy may help avoid unnecessary surgical intervention. Further studies with extended follow-up are needed to optimize diagnostic and therapeutic strategies.
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Affiliation(s)
- Nicole Hawkins
- General Surgery, Townsville University Hospital, Townsville, AUS
| | - David Sun
- General Surgery, Townsville University Hospital, Townsville, AUS
| | - Panuwat Pornkul
- General Surgery, Townsville University Hospital, Townsville, AUS
| | - Kaeun Bae
- General Surgery, Townsville University Hospital, Townsville, AUS
| | - Matan Ben David
- Upper Gastrointestinal and Hepatobiliary Surgery, Townsville University Hospital, Townsville, AUS
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Strainiene S, Sedleckaite K, Jarasunas J, Savlan I, Stanaitis J, Stundiene I, Strainys T, Liakina V, Valantinas J. Complicated course of biliary inflammatory myofibroblastic tumor mimicking hilar cholangiocarcinoma: A case report and literature review. World J Clin Cases 2021; 9:6155-6169. [PMID: 34368338 PMCID: PMC8316968 DOI: 10.12998/wjcc.v9.i21.6155] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2021] [Revised: 04/30/2021] [Accepted: 05/20/2021] [Indexed: 02/06/2023] Open
Abstract
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.
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Affiliation(s)
- Sandra Strainiene
- Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
| | | | - Juozas Jarasunas
- Department of Radiology, Nuclear Medicine and Medical Physics, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
| | - Ilona Savlan
- Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
| | - Juozas Stanaitis
- Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
| | - Ieva Stundiene
- Clinic of Gastroenterology, Nephrourology and Surgery, 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
- Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
- Department of Chemistry and Bioengineering, Faculty of Fundamental Science, Vilnius Gediminas Technical University, Vilnius 10223, Lithuania
| | - Jonas Valantinas
- Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Vilnius University, Vilnius 01513, Lithuania
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El-Diasty MT, Wazzan MA, Abduljabbar AH. Inflammatory Myofibroblastic Tumor of the Porta Hepatis: A Case Report. J Clin Imaging Sci 2021; 11:28. [PMID: 34221637 PMCID: PMC8247711 DOI: 10.25259/jcis_91_2021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2021] [Accepted: 05/09/2021] [Indexed: 11/04/2022] Open
Abstract
A 43-year-old man presented with painless jaundice. Imaging revealed a porta hepatis mass compressing the common bile duct. Endoscopic biopsy was negative for malignancy. Complete surgical resection was performed. Pathological assessment showed IGg4 negative inflammatory myofibroblastic tumor.
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Shah PA, Babu R, Ramaswamy V, G SK. Inflammatory myofibroblastic tumours of the duodenopancreaticobiliary tract. BMJ Case Rep 2021; 14:e240833. [PMID: 33958358 PMCID: PMC8103835 DOI: 10.1136/bcr-2020-240833] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/27/2021] [Indexed: 11/03/2022] Open
Abstract
Inflammatory myofibroblastic tumours (IMTs) are rare benign neoplasms of intermediate malignant potential that are found in the lungs and rarely at extrapulmonary sites common in children and young adults. IMTs tend to be locally invasive and have some amount of metastatic potential as well. We present two cases of IMTs involving the duodenum, pancreas and distal bile duct. The first case presented with extensive involvement of the first three parts of the duodenum and head of the pancreas, while the second presented with a pancreatic and biliary tree involvement. Upon examinations and investigations, these tumours mimicked malignant neoplasms. A Whipple procedure for surgical resection was undertaken in both cases. The histological findings showed fascicles of spindle cells with infiltration of lymphocytes and plasma cells. The inflammatory myofibroblastic tumour was diagnosed based on pathological grounds with immunohistochemistry. Preoperative diagnosis of IMTs is difficult and complete surgical resection is the primary treatment.
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Affiliation(s)
- Pritik A Shah
- Department of Hepato Pancreato Biliary(HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
| | - Raghavendra Babu
- Department of Hepato Pancreato Biliary(HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
| | - Veena Ramaswamy
- Department of Histopathology, Strand Life Sciences, Bangalore, Karnataka, India
| | - Subash K G
- Department of Hepato Pancreato Biliary(HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
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Shi X, Zhang T, Xu H, Zhang X, Zhao H, Liu X, Hou F, Yang G, Liu Z. Jaundice may be the only clinical manifestation of primary hepatosplenic diffuse large B-cell lymphoma: a case report and literature review. J Int Med Res 2020; 48:300060520938173. [PMID: 32762403 PMCID: PMC7416148 DOI: 10.1177/0300060520938173] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023] Open
Abstract
A 64-year old Chinese male patient was admitted to our hospital because of severe
jaundice that persisted for 2 months. No swollen lymph nodes or hepatosplenomegaly was
detected on physical examination. His laboratory data indicated high levels of direct
bilirubin, alkaline phosphatase, aspartate aminotransferase, and alanine aminotransferase.
No abnormality was revealed on abdominal computed tomography (CT). However, positron
emission tomography (PET)-CT revealed diffuse hypermetabolism in the liver and spleen.
Ultimately, liver biopsy guided by PET-CT was performed, revealing that atypical
lymphocytes diffusely infiltrated the liver. The immunohistochemical analysis demonstrated
that the tumor cells were positive for CD20, Bcl-2, Bcl-6, MUM1, and c-Myc but negative
for CD3, CD4, CD8, and CD10. Based on these findings, this patient was diagnosed with
primary hepatosplenic diffuse large B-cell lymphoma. After the definite diagnosis, he
received chemotherapy and remained in good health as of September 2019.
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Affiliation(s)
- Xue Shi
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Tingting Zhang
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Hong Xu
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xiaoying Zhang
- Department of Emergency, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Hongguo Zhao
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xiaodan Liu
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Fang Hou
- Department of Hematology Research Laboratory, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Guangjie Yang
- Department of PET/CT Center, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Zhihe Liu
- Department of Lymphoma, The Affiliated Hospital of Qingdao University, Qingdao, China
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Shatveryan GA, Bagmet NN, Ratnikova NP, Chardarov NK, Hrustaleva MV, Dolzhansky OV, Hovrin VV, Galyan TN. [Inflammatory myofibroblastic tumor of common bile duct]. Khirurgiia (Mosk) 2018:51-54. [PMID: 29992927 DOI: 10.17116/hirurgia2018751] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Affiliation(s)
- G A Shatveryan
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - N N Bagmet
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - N P Ratnikova
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - N K Chardarov
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - M V Hrustaleva
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - O V Dolzhansky
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - V V Hovrin
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
| | - T N Galyan
- Petrovsky Russian Research Center for Surgery, Moscow, Russia
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Pennington TE, Ozmen J, Fenton-Lee D. Torsion of omental fibrous pseudotumour mimicking acute appendicitis. J Surg Case Rep 2016; 2016:rjv170. [PMID: 26811304 PMCID: PMC4724786 DOI: 10.1093/jscr/rjv170] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
Unusual pathologies are occasionally found at laparoscopy when appendicitis is suspected. We present a case of strangulated inflammatory fibrous pseudotumour of the omentum presenting in a similar fashion to appendicitis. The infarcted omentum was excised, facilitating prompt resolution of symptoms.
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Affiliation(s)
| | - John Ozmen
- St. Vincent's Hospital, Darlinghurst, NSW, Australia
| | - Douglas Fenton-Lee
- St. Vincent's Hospital, Darlinghurst, NSW, Australia University of Notre Dame Australia, Darlinghurst, NSW, Australia
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Mid common bile duct inflammatory pseudotumor mimicking cholangiocarcinoma. A case report and literature review. Int J Surg Case Rep 2013; 5:12-5. [PMID: 24394855 PMCID: PMC3907201 DOI: 10.1016/j.ijscr.2013.10.017] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2013] [Revised: 10/29/2013] [Accepted: 10/29/2013] [Indexed: 01/03/2023] Open
Abstract
INTRODUCTION Biliary inflammatory pseudotumors (IPTs) represent an exceptional benign cause of obstructive jaundice. These lesions are often mistaken for cholangiocarcinomas and are treated with major resections, because their final diagnosis can be achieved only after formal pathological examination of the resected specimen. Consequently, biliary IPTs are usually managed with unnecessary major resections. PRESENTATION OF CASE A 71-year-old female patient underwent an extra-hepatic bile duct resection en-bloc with the gallbladder and regional lymph nodes for an obstructing intraluminal growing tumor of the mid common bile duct (CBD). Limited resection was decided intraoperatively because of negative for malignancy fast frozen sections analysis in addition to the benign macroscopic features of the lesion. Histologically the tumor proved an IPT, arising from the bile duct epithelium, composed of inflammatory cells and reactive mesenchymal tissues. DISCUSSION The present case underlines the value of intraoperative reassessment of patients undergoing surgical resection for histopathologically undiagnosed biliary occupying lesions, in order to optimize their surgical management. CONCLUSION The probability of benign lesions mimicking cholangiocarcinoma should always be considered to avoid unnecessary major surgical resections, especially in fragile and/or elderly patients.
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