title: Intraduct papillary mucinous neoplasm of the pancreas: a tumour linked with IgG4-related disease?
abstract: OBJECTIVE
  Intraduct papillary mucinous neoplasm (IPMN) is a pancreatic tumour that is often associated with chronic pancreatitis (CP) in the surrounding pancreas. Type 1 autoimmune pancreatitis (AIP) is a fibro-inflammatory condition with characteristic histological features and prominent IgG4+ plasma cells and is part of the spectrum of IgG4-related disease (IgG4-RD). The aim of this study was to determine whether CP associated with pancreatic IPMN commonly represents AIP.
  
  
  METHODS
  We identified two consecutive 'index' cases of pancreatic IPMN during routine reporting in which the adjacent pancreas showed morphological features suggestive of AIP. These cases were investigated using the Boston criteria for the histopathological diagnosis of IgG4-RD and the HISORt criteria for the clinical and histopathological diagnosis of AIP. Using the same criteria, we proceeded to a clinical review of 12 extra cases of IPMN in which the tumour or the surrounding pancreas showed significant lymphoplasmacytic inflammation and/or fibrosis.
  
  
  RESULTS
  Both of the 'index' cases fulfilled the HISORt criteria for AIP and both had morphological features characteristic of IgG4-RD using the Boston criteria, although only one possessed features 'highly suggestive of IgG4-RD' after immunohistochemistry. Additionally, both 'index' cases had radiological features that could represent extrapancreatic manifestations. Review of the 12 additional cases of IPMN revealed no further examples showing co-existent AIP.
  
  
  CONCLUSIONS
  While pancreatic IPMN and AIP may co-exist, most CP associated with IPMN does not represent AIP.
authors.affiliation: Department of Cellular Pathology, University Hospital Southampton NHS Foundation Trust, Southampton, UK. adrian.bateman@uhs.nhs.uk
authors.forename: Adrian C
authors.initials: AC
authors.lastname: Bateman
authors.forename: Emma L
authors.initials: EL
authors.lastname: Culver
authors.forename: Matthew
authors.initials: M
authors.lastname: Sommerlad
authors.forename: Runjan
authors.initials: R
authors.lastname: Chetty
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000150:complications / Q000473:pathology*; D000231:Adenocarcinoma, Papillary / Q000150:complications / Q000473:pathology*; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology*; D021441:Carcinoma, Pancreatic Ductal / Q000150:complications / Q000473:pathology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology*; D050500:Pancreatitis, Chronic / Q000150:complications / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G
keywords: INFLAMMATION; PANCREAS; PANCREATIC CANCER