Follicular cholangitis mimicking a common bile duct cancer: a case report

Abstract Background Follicular cholangitis (FC) is a benign bile duct disease that was first reported 2003. Pathologically, it is characterized by lymphoplasmacytic infiltration with multiple lymphoid follicle formations under the mucosal layer of the biliary tract. However, as this disease is extre...

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Main Authors: Kenji Koneri, Takanori Goi, Hokahiro Katayama, Noriyuki Tagai, Michiaki Shimada, Hidetaka Kurebayashi, Katsuji Sawai, Mitsuhiro Morikawa, Masato Tamaki, Yasuo Hirono, Satomi Hatta, Yoshiaki Imamura, Makoto Murakami
Format: Article
Language:English
Published: Japan Surgical Society 2023-07-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-023-01708-6
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author Kenji Koneri
Takanori Goi
Hokahiro Katayama
Noriyuki Tagai
Michiaki Shimada
Hidetaka Kurebayashi
Katsuji Sawai
Mitsuhiro Morikawa
Masato Tamaki
Yasuo Hirono
Satomi Hatta
Yoshiaki Imamura
Makoto Murakami
author_facet Kenji Koneri
Takanori Goi
Hokahiro Katayama
Noriyuki Tagai
Michiaki Shimada
Hidetaka Kurebayashi
Katsuji Sawai
Mitsuhiro Morikawa
Masato Tamaki
Yasuo Hirono
Satomi Hatta
Yoshiaki Imamura
Makoto Murakami
author_sort Kenji Koneri
collection DOAJ
description Abstract Background Follicular cholangitis (FC) is a benign bile duct disease that was first reported 2003. Pathologically, it is characterized by lymphoplasmacytic infiltration with multiple lymphoid follicle formations under the mucosal layer of the biliary tract. However, as this disease is extremely rare, little is known about its etiology and pathogenesis. Case presentation A 77-year-old woman was diagnosed with middle bile duct stenosis and potential increases in alkaline phosphatase (ALP) and γ-glutamyl transpeptidase levels (γ-GTP). Carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9) and IgG4 levels were all within the normal limits. Contrast-enhanced computed tomography (CE-CT) and magnetic resonance imaging (MRI) revealed bile duct dilation from intrahepatic to upper common bile duct and an irregular mass lesion in distal bile duct. Additionally, multiple overlapping leaf-like folds were detected. 18F-fluorodeoxyglucose positron emission tomography–computed tomography (18F-FDG-PET/CT) did not demonstrate fluorodeoxyglucose uptake. Subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection was performed because common bile duct cancer could not be ruled out. The resected specimen showed diffuse homogeneous middle bile duct wall thickening. Microscopically, the lesion exhibited thick fibrosis with several invaded lymphoplasmacytic cells, and lymphoid follicle formations were detected under the mucosal layer. Immunohistochemical staining (IHC) revealed positive for CD3, CD4, CD20 and CD79a, and these findings led to a final diagnosis of FC. The patient has not experienced recurrence to date (42 months postoperatively). Conclusions Currently, accurate preoperative diagnosis of FC is difficult. More cases must be accumulated to generate additional knowledge on its precise diagnosis and proper treatment.
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publishDate 2023-07-01
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spelling doaj-art-7cc1df1db22a4e78bacc8ed9f5df19dc2025-08-20T03:56:59ZengJapan Surgical SocietySurgical Case Reports2198-77932023-07-01911810.1186/s40792-023-01708-6Follicular cholangitis mimicking a common bile duct cancer: a case reportKenji Koneri0Takanori Goi1Hokahiro Katayama2Noriyuki Tagai3Michiaki Shimada4Hidetaka Kurebayashi5Katsuji Sawai6Mitsuhiro Morikawa7Masato Tamaki8Yasuo Hirono9Satomi Hatta10Yoshiaki Imamura11Makoto Murakami12First Department of Surgery, University of FukuiFirst Department of Surgery, University of FukuiDepartment of Surgery, Tsukushino HospitalFirst Department of Surgery, University of FukuiFirst Department of Surgery, University of FukuiFirst Department of Surgery, University of FukuiFirst Department of Surgery, University of FukuiFirst Department of Surgery, University of FukuiFirst Department of Surgery, University of FukuiCancer Care Portion Center, University of FukuiDivision of Diagnostic Pathology/Surgical Pathology, University of FukuiDivision of Diagnostic Pathology/Surgical Pathology, University of FukuiFirst Department of Surgery, University of FukuiAbstract Background Follicular cholangitis (FC) is a benign bile duct disease that was first reported 2003. Pathologically, it is characterized by lymphoplasmacytic infiltration with multiple lymphoid follicle formations under the mucosal layer of the biliary tract. However, as this disease is extremely rare, little is known about its etiology and pathogenesis. Case presentation A 77-year-old woman was diagnosed with middle bile duct stenosis and potential increases in alkaline phosphatase (ALP) and γ-glutamyl transpeptidase levels (γ-GTP). Carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9) and IgG4 levels were all within the normal limits. Contrast-enhanced computed tomography (CE-CT) and magnetic resonance imaging (MRI) revealed bile duct dilation from intrahepatic to upper common bile duct and an irregular mass lesion in distal bile duct. Additionally, multiple overlapping leaf-like folds were detected. 18F-fluorodeoxyglucose positron emission tomography–computed tomography (18F-FDG-PET/CT) did not demonstrate fluorodeoxyglucose uptake. Subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection was performed because common bile duct cancer could not be ruled out. The resected specimen showed diffuse homogeneous middle bile duct wall thickening. Microscopically, the lesion exhibited thick fibrosis with several invaded lymphoplasmacytic cells, and lymphoid follicle formations were detected under the mucosal layer. Immunohistochemical staining (IHC) revealed positive for CD3, CD4, CD20 and CD79a, and these findings led to a final diagnosis of FC. The patient has not experienced recurrence to date (42 months postoperatively). Conclusions Currently, accurate preoperative diagnosis of FC is difficult. More cases must be accumulated to generate additional knowledge on its precise diagnosis and proper treatment.https://doi.org/10.1186/s40792-023-01708-6Follicular cholangitisDistal common bile ductCommon bile duct cancerCase report
spellingShingle Kenji Koneri
Takanori Goi
Hokahiro Katayama
Noriyuki Tagai
Michiaki Shimada
Hidetaka Kurebayashi
Katsuji Sawai
Mitsuhiro Morikawa
Masato Tamaki
Yasuo Hirono
Satomi Hatta
Yoshiaki Imamura
Makoto Murakami
Follicular cholangitis mimicking a common bile duct cancer: a case report
Surgical Case Reports
Follicular cholangitis
Distal common bile duct
Common bile duct cancer
Case report
title Follicular cholangitis mimicking a common bile duct cancer: a case report
title_full Follicular cholangitis mimicking a common bile duct cancer: a case report
title_fullStr Follicular cholangitis mimicking a common bile duct cancer: a case report
title_full_unstemmed Follicular cholangitis mimicking a common bile duct cancer: a case report
title_short Follicular cholangitis mimicking a common bile duct cancer: a case report
title_sort follicular cholangitis mimicking a common bile duct cancer a case report
topic Follicular cholangitis
Distal common bile duct
Common bile duct cancer
Case report
url https://doi.org/10.1186/s40792-023-01708-6
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