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A rare cause of obstructive jaundice: Is surgery the only option?
Journal Pre-proof A rare cause of obstructive jaundice: Is surgery the only option? Eun Shin, M.D., Se Woo Park, M.D., Ph.D.
PII: DOI: Reference:
S0016-5085(20)30091-3 https://doi.org/10.1053/j.gastro.2019.11.307 YGAST 63142
To appear in: Gastroenterology Accepted Date: 5 November 2019 Please cite this article as: Shin E, Park SW, A rare cause of obstructive jaundice: Is surgery the only option?, Gastroenterology (2020), doi: https://doi.org/10.1053/j.gastro.2019.11.307. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 by the AGA Institute
A rare cause of obstructive jaundice: Is surgery the only option?
Authors: Eun Shin, M.D.1, Se Woo Park, M.D., Ph.D. 2
Departmental and institutional affiliations: 1
Department of Pathology, Hallym University Dongtan Sacred Heart Hospital, Hallym
University College of Medicine, Gyeonggi-do, Korea 2
Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan
Sacred Heart Hospital, Hallym University College of Medicine, Gyeonggi-do, Korea
Correspondence to: Professor Se Woo Park, M.D., Ph.D. Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, 7, Keunjaebong-gil, Hwaseong-si, Gyeonggi-do, 18450, Korea Tel: +82-31-8086-2858, Fax: +82-31-8086-2029, E-mail: [email protected]
Authorship Guarantor of the article: Se Woo Park Specific author contributions: Se Woo Park conceived and designed the study. Se Woo Park and Eun Shin provided study materials or patients. Se Woo Park and Eun Shin analyzed and interpreted the data. Se Woo Park and Eun Shin drafted the article. Se Woo Park critically revised the article for important intellectual content. All authors were responsible for conducting the study, including care of the patients, acquisition of the data, and interpretation of the data after analysis. After drafting of the initial manuscript, all authors critically revised the manuscript and approved the final version.
Disclosures Se Woo Park and Eun Shin have no conflicts of interest or financial ties to disclose.
Question: A 71-old men presented with a 2-week history of painless jaundice. Physical examination showed icteric sclera and abdominal examination revealed slight deep tenderness in epigastrium. Laboratory tests showed the following results: aminotransferase (AST), 251 IU/L; alanine transaminase (ALT), 118 IU/L; serum total bilirubin, 4.0 mg/dL and carbohydrate antigen (CA) 19-9, 50.0 U/mL. Also, serum total IgG revealed 1380.90 mg/dL with 135.2mg/dL of IgG4 subclass. Abdominal computed tomography (CT) revealed a relatively well-defined mass with homogeneous density in pancreatic head causing bile and pancreatic duct dilatation (Figure 1). The positron emission tomography (PET) CT revealed about 5cm focal abnormal accumulation of fluorodeoxyglucose (FDG) in pancreatic head portion and periportal lymph node (Figure 2). Endoscopic ultrasonography (EUS) revealed a heterogeneous low echogenic mass in pancreatic head with multifocal hyperechoic foci and marked bile duct dilatation with ductal invasion (Figure 3). EUS-guided tissue acquisition and endoscopic retrograde cholangio-pancreatography was performed. The cholangiography shows short segment stricture at distal common bile duct with upstream ductal dilatation (Figure 4), subsequently, decompression with plastic stent placement for obstructive jaundice was done. Histologic findings from EUS-guided tissue acquisition and endo-biliary biopsy revealed abundant infiltration of IgG4 positive plasma cells and lymphocytes around the pancreatic duct with storiform fibrosis (Figure 5 & 6). Based on the patient’s clinical history, presentation, and objective findings, what is the most likely diagnosis and appropriate management? A. Pancreatic ductal adenocarcinoma, pancreaticoduodenectomy B. Pancreatic neuroendocrine tumor, pancreaticoduodenectomy C. Mass-forming chronic pancreatitis, conservative treatment D. Mass-forming IgG4 related autoimmune pancreatitis, steroid therapy
Answer:
Focal
mass-forming
IgG4
related
autoimmune
pancreatitis
(AIP)
masquerading as pancreatic cancer The correct answer is D. In our case, the patient presented with painless jaundice and radiologic findings revealed a mass in the pancreatic head with bile duct obstruction. Also, PET CT revealed a swollen head of the pancreas with focal intense FDG uptake, which is suspicious for malignancy. In this regard, a surgical resection to differentiate pancreatic malignancy would be reasonable. However, when we reviewed this case, the following questions deserved careful concerns: according to several criteria, typical radiologic findings of IgG4 related AIP included a diffuse sausage like parenchymal enlargement with peripancreatic radiolucent halo and multi-focal strictures in main pancreatic duct.1 In our case, pancreatic head mass presented delayed homogenous enhancement which can be a differential point between focal mass-forming IgG4 related AIP and pancreatic cancer.2 Furthermore, careful consideration has to be allowed when
serum IgG4 was taken as a
specific marker for a differential diagnosis between AIP and pancreatic cancer. According to recent studies, the cutoff value of serum IgG4 concentration was independently confirmed as 135 mg/ dL3 while serum IgG4 was marginally elevated as 135.2mg/dL which is not sufficient for an AIP diagnosis in our case. In addition, the decision for diagnostic steroid treatments should not be made until the possibility of pancreatic cancer is excluded through three times of EUS-guided tissue acquisition in our case. This taught us a lesson that for patients for whom AIP is suspected, enough tissue sampling should be addressed before steroid trial. Truly, he was managed with high dose steroid for 4 weeks and gradual tapering, with complete improvement in symptom and radiological findings (Figure 7 & 8) on followup over a period of 6 months. Although AIP is a benign disease, it is commonly misdiagnosed as a malignancy. Clinicians should increase their awareness of AIP to avoid
further misdiagnoses of cancer and unnecessary surgery. Furthermore, early steroid therapy without a clear pathologic confirm is not recommended.
References
1.
Shimosegawa T, Chari ST, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 2011;40:352-8.
2.
Sun GF, Zuo CJ, Shao CW, et al. Focal autoimmune pancreatitis: radiological characteristics help to distinguish from pancreatic cancer. World J Gastroenterol 2013;19:3634-41.
3.
Okazaki K, Umehara H. Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan. Int J Rheumatol 2012;2012:357071.
PII: DOI: Reference:
S0016-5085(20)30091-3 https://doi.org/10.1053/j.gastro.2019.11.307 YGAST 63142
To appear in: Gastroenterology Accepted Date: 5 November 2019 Please cite this article as: Shin E, Park SW, A rare cause of obstructive jaundice: Is surgery the only option?, Gastroenterology (2020), doi: https://doi.org/10.1053/j.gastro.2019.11.307. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 by the AGA Institute
A rare cause of obstructive jaundice: Is surgery the only option?
Authors: Eun Shin, M.D.1, Se Woo Park, M.D., Ph.D. 2
Departmental and institutional affiliations: 1
Department of Pathology, Hallym University Dongtan Sacred Heart Hospital, Hallym
University College of Medicine, Gyeonggi-do, Korea 2
Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan
Sacred Heart Hospital, Hallym University College of Medicine, Gyeonggi-do, Korea
Correspondence to: Professor Se Woo Park, M.D., Ph.D. Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, 7, Keunjaebong-gil, Hwaseong-si, Gyeonggi-do, 18450, Korea Tel: +82-31-8086-2858, Fax: +82-31-8086-2029, E-mail: [email protected]
Authorship Guarantor of the article: Se Woo Park Specific author contributions: Se Woo Park conceived and designed the study. Se Woo Park and Eun Shin provided study materials or patients. Se Woo Park and Eun Shin analyzed and interpreted the data. Se Woo Park and Eun Shin drafted the article. Se Woo Park critically revised the article for important intellectual content. All authors were responsible for conducting the study, including care of the patients, acquisition of the data, and interpretation of the data after analysis. After drafting of the initial manuscript, all authors critically revised the manuscript and approved the final version.
Disclosures Se Woo Park and Eun Shin have no conflicts of interest or financial ties to disclose.
Question: A 71-old men presented with a 2-week history of painless jaundice. Physical examination showed icteric sclera and abdominal examination revealed slight deep tenderness in epigastrium. Laboratory tests showed the following results: aminotransferase (AST), 251 IU/L; alanine transaminase (ALT), 118 IU/L; serum total bilirubin, 4.0 mg/dL and carbohydrate antigen (CA) 19-9, 50.0 U/mL. Also, serum total IgG revealed 1380.90 mg/dL with 135.2mg/dL of IgG4 subclass. Abdominal computed tomography (CT) revealed a relatively well-defined mass with homogeneous density in pancreatic head causing bile and pancreatic duct dilatation (Figure 1). The positron emission tomography (PET) CT revealed about 5cm focal abnormal accumulation of fluorodeoxyglucose (FDG) in pancreatic head portion and periportal lymph node (Figure 2). Endoscopic ultrasonography (EUS) revealed a heterogeneous low echogenic mass in pancreatic head with multifocal hyperechoic foci and marked bile duct dilatation with ductal invasion (Figure 3). EUS-guided tissue acquisition and endoscopic retrograde cholangio-pancreatography was performed. The cholangiography shows short segment stricture at distal common bile duct with upstream ductal dilatation (Figure 4), subsequently, decompression with plastic stent placement for obstructive jaundice was done. Histologic findings from EUS-guided tissue acquisition and endo-biliary biopsy revealed abundant infiltration of IgG4 positive plasma cells and lymphocytes around the pancreatic duct with storiform fibrosis (Figure 5 & 6). Based on the patient’s clinical history, presentation, and objective findings, what is the most likely diagnosis and appropriate management? A. Pancreatic ductal adenocarcinoma, pancreaticoduodenectomy B. Pancreatic neuroendocrine tumor, pancreaticoduodenectomy C. Mass-forming chronic pancreatitis, conservative treatment D. Mass-forming IgG4 related autoimmune pancreatitis, steroid therapy
Answer:
Focal
mass-forming
IgG4
related
autoimmune
pancreatitis
(AIP)
masquerading as pancreatic cancer The correct answer is D. In our case, the patient presented with painless jaundice and radiologic findings revealed a mass in the pancreatic head with bile duct obstruction. Also, PET CT revealed a swollen head of the pancreas with focal intense FDG uptake, which is suspicious for malignancy. In this regard, a surgical resection to differentiate pancreatic malignancy would be reasonable. However, when we reviewed this case, the following questions deserved careful concerns: according to several criteria, typical radiologic findings of IgG4 related AIP included a diffuse sausage like parenchymal enlargement with peripancreatic radiolucent halo and multi-focal strictures in main pancreatic duct.1 In our case, pancreatic head mass presented delayed homogenous enhancement which can be a differential point between focal mass-forming IgG4 related AIP and pancreatic cancer.2 Furthermore, careful consideration has to be allowed when
serum IgG4 was taken as a
specific marker for a differential diagnosis between AIP and pancreatic cancer. According to recent studies, the cutoff value of serum IgG4 concentration was independently confirmed as 135 mg/ dL3 while serum IgG4 was marginally elevated as 135.2mg/dL which is not sufficient for an AIP diagnosis in our case. In addition, the decision for diagnostic steroid treatments should not be made until the possibility of pancreatic cancer is excluded through three times of EUS-guided tissue acquisition in our case. This taught us a lesson that for patients for whom AIP is suspected, enough tissue sampling should be addressed before steroid trial. Truly, he was managed with high dose steroid for 4 weeks and gradual tapering, with complete improvement in symptom and radiological findings (Figure 7 & 8) on followup over a period of 6 months. Although AIP is a benign disease, it is commonly misdiagnosed as a malignancy. Clinicians should increase their awareness of AIP to avoid
further misdiagnoses of cancer and unnecessary surgery. Furthermore, early steroid therapy without a clear pathologic confirm is not recommended.
References
1.
Shimosegawa T, Chari ST, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 2011;40:352-8.
2.
Sun GF, Zuo CJ, Shao CW, et al. Focal autoimmune pancreatitis: radiological characteristics help to distinguish from pancreatic cancer. World J Gastroenterol 2013;19:3634-41.
3.
Okazaki K, Umehara H. Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan. Int J Rheumatol 2012;2012:357071.