Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report

Abstract Background Autoimmune pancreatitis (AIP) is a rare disease that manifests as pancreatic involvement in systemic IgG4-related disease (IgG4-RD), a special type of chronic pancreatitis caused by autoimmune abnormalities. The main imaging manifestations of IgG4-related AIP consist of diffuse o...

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Autores principales: Han Wang, Lan Yao, Ligang Wang, Xixi Sun, Bin Huang
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Publicado: BMC 2021
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spelling oai:doaj.org-article:e3a6fab6101240e589a418f2c2acfed62021-11-14T12:16:47ZFocal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report10.1186/s12876-021-01996-y1471-230Xhttps://doaj.org/article/e3a6fab6101240e589a418f2c2acfed62021-11-01T00:00:00Zhttps://doi.org/10.1186/s12876-021-01996-yhttps://doaj.org/toc/1471-230XAbstract Background Autoimmune pancreatitis (AIP) is a rare disease that manifests as pancreatic involvement in systemic IgG4-related disease (IgG4-RD), a special type of chronic pancreatitis caused by autoimmune abnormalities. The main imaging manifestations of IgG4-related AIP consist of diffuse or localized pancreatic enlargement and irregular pancreatic duct narrowing. The diagnosis of AIP is challenging because it can present with focal lesions, similar to radiologically bile duct cancer or pancreatic cancer. Case presentation A 55-year-old male patient was admitted with painless jaundice and multiple radiographic findings of pancreatic head mass, as well as intrahepatic and extrahepatic bile duct dilatation. Various imaging methods indicated pancreatic cancer. However, the endoscopic ultrasonography guided fine needle aspiration (EUS-FNA) and a laparoscopic pancreatic biopsy suggested an IgG4-related AIP. After one month, magnetic resonance imaging showed that the lesion had slightly grown. Combined with CA19-9 and other indexes, the possibility of malignancy was high and there were still surgical indications. The pathological analysis following a pancreaticoduodenectomy revealed poorly differentiated adenocarcinoma in the distal common bile duct. Conclusion To date, few reports have described pancreatic or extrapancreatic malignancies in AIP patients, and no association between AIP and bile duct adenocarcinoma has been previously confirmed. This case discuss the differentiation between AIP and malignancy, recent research progress, and the correlation between the two diseases, highlights the importance of carefully evaluating patients with AIP to rule out potential tumors, as well as the critical need for follow up treatment.Han WangLan YaoLigang WangXixi SunBin HuangBMCarticleIgG4Autoimmune pancreatitisCarcinoma of bile ductIgG4-related diseaseCase reportDiseases of the digestive system. GastroenterologyRC799-869ENBMC Gastroenterology, Vol 21, Iss 1, Pp 1-8 (2021)
institution DOAJ
collection DOAJ
language EN
topic IgG4
Autoimmune pancreatitis
Carcinoma of bile duct
IgG4-related disease
Case report
Diseases of the digestive system. Gastroenterology
RC799-869
spellingShingle IgG4
Autoimmune pancreatitis
Carcinoma of bile duct
IgG4-related disease
Case report
Diseases of the digestive system. Gastroenterology
RC799-869
Han Wang
Lan Yao
Ligang Wang
Xixi Sun
Bin Huang
Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
description Abstract Background Autoimmune pancreatitis (AIP) is a rare disease that manifests as pancreatic involvement in systemic IgG4-related disease (IgG4-RD), a special type of chronic pancreatitis caused by autoimmune abnormalities. The main imaging manifestations of IgG4-related AIP consist of diffuse or localized pancreatic enlargement and irregular pancreatic duct narrowing. The diagnosis of AIP is challenging because it can present with focal lesions, similar to radiologically bile duct cancer or pancreatic cancer. Case presentation A 55-year-old male patient was admitted with painless jaundice and multiple radiographic findings of pancreatic head mass, as well as intrahepatic and extrahepatic bile duct dilatation. Various imaging methods indicated pancreatic cancer. However, the endoscopic ultrasonography guided fine needle aspiration (EUS-FNA) and a laparoscopic pancreatic biopsy suggested an IgG4-related AIP. After one month, magnetic resonance imaging showed that the lesion had slightly grown. Combined with CA19-9 and other indexes, the possibility of malignancy was high and there were still surgical indications. The pathological analysis following a pancreaticoduodenectomy revealed poorly differentiated adenocarcinoma in the distal common bile duct. Conclusion To date, few reports have described pancreatic or extrapancreatic malignancies in AIP patients, and no association between AIP and bile duct adenocarcinoma has been previously confirmed. This case discuss the differentiation between AIP and malignancy, recent research progress, and the correlation between the two diseases, highlights the importance of carefully evaluating patients with AIP to rule out potential tumors, as well as the critical need for follow up treatment.
format article
author Han Wang
Lan Yao
Ligang Wang
Xixi Sun
Bin Huang
author_facet Han Wang
Lan Yao
Ligang Wang
Xixi Sun
Bin Huang
author_sort Han Wang
title Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
title_short Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
title_full Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
title_fullStr Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
title_full_unstemmed Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
title_sort focal igg4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report
publisher BMC
publishDate 2021
url https://doaj.org/article/e3a6fab6101240e589a418f2c2acfed6
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AT lanyao focaligg4relatedautoimmunepancreatitiswithdistalcholedochaladenocarcinomaararecasereport
AT ligangwang focaligg4relatedautoimmunepancreatitiswithdistalcholedochaladenocarcinomaararecasereport
AT xixisun focaligg4relatedautoimmunepancreatitiswithdistalcholedochaladenocarcinomaararecasereport
AT binhuang focaligg4relatedautoimmunepancreatitiswithdistalcholedochaladenocarcinomaararecasereport
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