Malignant islet cell tumor projecting into the main pancreatic duct.

We report herein a rare case of islet cell tumor showing a unique growth pattern in a patient who developed repeated acute pancreatitis as the tumor's initial symptom. Preoperative imaging examinations showed dilatation of the main pancreatic duct (MPD) and cysts around the pancreatic tail. A distal pancreatectomy with splenectomy was performed because the pancreatitis was localized in the distal pancreas and was not controlled by various drug therapies. Grossly, the tumor consisted of two component parts: a markedly infiltrative part in the pancreatic parenchyma, and a papillary elevated part in the MPD. The MPD was obstructed by the tumor spreading widely along the distal MPD. Microscopically, the tumor was composed entirely of islet cell tumors (nonfunctioning), with several foci of venous and lymphatic involvement. Based on its growth behavior, we assumed that the tumor may have arisen from the MPD or from islet cells closely adjacent to the MPD. The patient's postoperative course was uneventful and he is doing well 2 years after the operation. We discuss the growth pattern of the tumor and the cause of the pancreatitis.