Three recent topics in autoimmune pancreatitis were described. First is about the revised clinical diagnostic criteria of autoimmune pancreatitis proposed in 2006. In this new criteria, cases showing localized ductal narrowing over less than one-third the length of the pancreas can be diagnosed, and serological findings showing elevation of the serum IgG4 level is included as one diagnostic factor. Second is about autoimmune pancreatitis with neutrophilic infiltration in the epithelium of the pancreatic duct that was reported by American and Italian pathologists. These patients showed different clinicopathological features from autoimmune pancreatitis defined in Japan as follows: no prediction for elderly males, frequent association with inflammatory bowel disease, and weaker association with other sclerosing diseases. Third is about proposal of a novel clinicopathological entity, an IgG4-related sclerosing disease that is characterized by extensive IgG4-positive plasma cell and T-lymphocyte infiltration of various organs. Autoimmune pancreatitis might be not simply a pancreatitis, but a pancreatic lesion of this systemic disease.

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Protein kinase C (PKC) is a family of serine/threonine kinases comprising 10 isoforms. Each PKC isoform has a different pattern of cell distribution, can be activated independently by specific stimuli, and mediates distinct biological functions. In pancreatic acinar cells, 4 PKC isoforms, -α, -δ, -ε, and -ζ, have been detected. PKC-δ is responsible for physiological amylase secretion, and PKC-δ and -ε are implicated in NF-κB activation, a transcription factor important in the inflammatory response during pancreatitis. PKC also mediates the critical events for pancreatitis such as the inhibition of digestive enzyme secretion, the disruption of actin cytoskeleton, and the cell death although the PKC isoforms involved are remain to be determined. Understanding the mechanisms by which PKC isoforms mediate the newer signal transduction pathways and their roles in acinar cell pathophysiology will be an important area for future investigation.

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The Patient was a 51-year-old woman with diabetes mellitus. Abdominal ultrasonography revealed two hypoechoic lesions in the pancreas body and tail. CT showed low density areas without enhancement. Serum hormones (insulin, gastrin, glucagon) were within normal limits. Based on a diagnosis of multiple non-functioning endocrine tumors or solid-pseudopapillary tumors without cystic degeneration, spleen preserving distal pancreatectomy was performed. Histopathologically, neoplastic cells with small nuclei were arranged in a trabecular form with hyaline degeneration. Both Grimelius argyrophil reaction and chromogranin A reaction were positive. The tumors were diagnosed histopathologically as non-functioning endocrine tumors with hyaline degeneration. There was also marked hyaline degeneration of most of the islet cells in the non-tumor region the pancreas.

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We report a case of pancreatic carcinoma in the pancreaticogastrostomy site after pylorus-preserving pancreatoduodenectomy (PPPD). A 57-year-old man who had undergone PPPD for intraductal papillary mucinous neoplasm (IPMN) of the pancreatic head 11 years before was admitted to our hospital for surgical treatment of gastric carcinoma at the pancreaticogastrostomy site. Reconstruction after the PPPD had been done by pacreaticogastrostomy, end-end duodenojejunostomy, and end-side choledochojejunostomy. The histopathological diagnosis had been papillary adenoma. We performed en bloc resection of the distal stomach, the remnant pancreas 3 cm from the junction of the pancreaticogastrostomy and a small part of the transverse colon, then reconstructed using Whipple's procedure. Histopathological examination showed moderately differentiated tubular adenocarcinoma of the remnant pancreas. Metachronous carcinogenesis in the pancreaticogastrostomy site after PPPD for IPMN is rare, and a very careful follow-up is necessary for a long period after the first operation.

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A 71-year-old man, who had been followed-up for chronic alcoholic pancreatitis, was admitted to the hospital under the diagnosis of sternoclavicular arthritis. Chest X-ray revealed left pleural effusion. Computed tomography demonstrated a pancreatic pseudocyst at the hilum of the spleen. ERCP demonstrated a villous tumor at the duodenal papilla, and the pathohistology of the biopsy specimens revealed adenocarcinoma. The main pancreatic duct was remarkably dilatated at the pancreatic head, and an inner filling defect was observed. PPPD was performed, and intraductal papillary-mucinous carcinoma was diagnosed pathologically. When a pancreatic pseudocyst is observed, it is necessary to consider the possibility of pancreatic cancer and an intraductal papillary-mucinous tumor (IPMT).

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A 61-year-old female with cystic lesions in the liver and pancreatic tail, which had been detected in 1992, was admitted to our hospital in 2005 because of a remarkably developed liver cystic lesion. Abdominal ultrasonography and CT scans showed unilocular clear-margin cystic lesions without a septum-like structure, a solid component, wall thicknening, and calcification in both the liver and pancreatic tail. However, because her previous CT findings showed that the clearly enhanced thickened wall of the pancreatic cystic lesion diminished annually with the lesion's increased diameter, we applied fenestration for the liver cystic lesion and, in contrast, distal pancreatectomy for the pancreatic lesion. Pathological findings revealed that the pancreatic cystic lesion was a mucinous cystadenoma with a typical ovarian-like stroma. Unilocular cystic lesions in the pancreatic tail may be mucinous cystic tumors even without the characteristic features suggesting a cystic tumor. Thus, reviewing past images can be useful for the diagnosis.

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We describe a 38-year-old woman with itching and eruption that had migrated from the lower legs to the upper body. The glucagon level was 2,100 pg/ml (upper limit of normal, 180 pg/ml). Computed tomographic scan of the abdomen revealed a 3.5 cm mass in the tail of the pancreas. The tumor was removed and found to be a glucagonoma. The clinical eruption resolved promptly and the glucagon level decreased to the normal range after surgical exision. The patient was alive with no recurrence 4 years after surgery.

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A 62-year-old man visited another hospital, complaining of general fatigue and jaundice. Based on imaging studies, the diagnosis of autoimmune pancreatitis accompanied by biliary stenosis was made. After endoscopic biliary stenting, oral prednisolone was started. Although the diffuse pancreas awelling and pancreatic duct narrowing improved, the biliary stenosis showed no improvement. The patient was admitted to our hospital for further investigations. Because the patient refused re-treatment with high-dose oral prednisolone, we administrated steroid mini-pulse therapy, resulting in an improvement of the biliary stenosis. Although oral prednisolone was prescribed as a maintenance treatment, and was gradually reduced, the patient has remained asymptomatic without elevations of hepatobiliary enzymes or biliary stenosis.

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