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Case Reports in Surgery
Volume 2013, Article ID 809023, 6 pages
Case Report

A Case of Pancreatic Cancer in the Setting of Autoimmune Pancreatitis with Nondiagnostic Serum Markers

1Hepatobiliary Unit, Royal Adelaide Hospital, North Terrace, Adelaide, SA 5000, Australia
2Department of Gastroenterology, Royal Adelaide Hospital, Adelaide, SA 5000, Australia
3Department of Surgical Pathology, SA Pathology, Adelaide, Australia
4Department of Gastroenterology, Flinders Medical Centre, Bedford Park, SA 5042, Australia

Received 9 April 2013; Accepted 15 May 2013

Academic Editors: G. Lal, N. D. Merrett, and S. Tatebe

Copyright © 2013 Manju D. Chandrasegaram et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Background. Autoimmune pancreatitis (AIP) often mimics pancreatic cancer. The diagnosis of both conditions is difficult preoperatively let alone when they coexist. Several reports have been published describing pancreatic cancer in the setting of AIP. Case Report. The case of a 53-year-old man who presented with abdominal pain, jaundice, and radiological features of autoimmune pancreatitis, with a “sausage-shaped” pancreas and bulky pancreatic head with portal vein impingement, is presented. He had a normal serum IgG4 and only mildly elevated Ca-19.9. Initial endoscopic ultrasound-(EUS-) guided fine-needle aspiration (FNA) of the pancreas revealed an inflammatory sclerosing process only. A repeat EUS guided biopsy following biliary decompression demonstrated both malignancy and features of autoimmune pancreatitis. At laparotomy, a uniformly hard, bulky pancreas was found with no sonographically definable mass. A total pancreatectomy with portal vein resection and reconstruction was performed. Histology revealed adenosquamous carcinoma of the pancreatic head and autoimmune pancreatitis and squamous metaplasia in the remaining pancreas. Conclusion. This case highlights the diagnostic and management difficulties in a patient with pancreatic cancer in the setting of serum IgG4-negative, Type 2 AIP.