The case was an 80-year-old male. He had been receiving treatment for diabetes for the past 20 years but was referred to this hospital for examination due to poor blood glucose control. Follow-up contrast-enhanced CT indicated a soft tissue shadow in the pancreatic head that was associated with a 27 mm multilocular cystic tumor, main pancreatic duct dilatation in the pancreatic body, and a 38 mm solid low-absorption mass with clear margins in the pancreatic tail. Contrast-enhanced endoscopic ultrasound (EUS) indicated nodules associated with contrast enhancement within the cysts of the cystic tumor in the pancreatic head. The resulting diagnosis was pancreatic tail cancer with concurrent intraductal papillary mucinous neoplasm (IPMN) in the pancreatic head and high-risk stigmata and total pancreatectomy was performed. The pathological diagnosis was IPMN for the lesion in the pancreatic head, and results of immunostaining of the tumor in the pancreatic tail were positive for trypsin and chymotrypsin, which led to a diagnosis of acinar cell carcinoma (ACC) . Although pancreatic duct cancer is commonly seen in cases of IPMN, cases in ACC complication as in the present case are extremely rare.
