ABSTRACT
Intraductal papillary mucinous tumor (IPMT) of the pancreas is a recently understood unique clinicopathologic disease entity comprising approximately 1% of all exocrine pancreatic tumors and 11% of cystic neoplasms of pancreas. It has been reported worldwide, mostly in Japan. It is generally characterized by recurrent pancreatitis, mucin oozing from the papilla of Vater, and dilated pancreatic duct with intraductal filling defects. Microscopically, the mucin-producing columnar epithelium forms papillary proliferation into the dilated pancreatic duct and this feature differentiates IPMT of the pancreas from the more common mucinous cystic neoplasms of the pancreas which usually do not communicate with the pancreatic duct. On the other hand, mucin-hypersecreting bile duct tumors have been rarely reported in the English literature. We herein present the first case of mucin-hypersecreting bile duct tumor combied with IPMT of the pancreas with a review of the related literature.