Pancreatic mass lesions associated with raised concentration of IgG4

Autoimmune pancreatitis (AIP) is a recognized benign disease characterized by irregular narrowing of the pancreatic duct, swelling of parenchyma, lymphoplasmacytic infiltration and fibrosis, and a favorable response to corticosteroid treatment. In this condition, the whole pancreas is diffusely affected. Recently, however, a few cases with locally affected lesions were reported, with some of them showing features similar to cancer. We reviewed 138 patients with pancreatic mass lesion, of which 17 were not initially diagnosed despite examinations. Serum IgG4 levels were elevated in seven of them. Their biopsy specimens had a similar appearance to those of AIP. We considered that they should be diagnosed as AIP or conditions related to AIP. Among the 10 patients without elevated IgG4, 4 patients were diagnosed as pancreatic cancer after follow-up, 1 presented with an islet cell tumor, 1 presented AIP with sclerosing cholangitis, and the other 4 had chronic pancreatitis.