Abstract

Intraductal tubulopapillary neoplasm is a rare primary pancreatic tumor that accounts for less than 1% of all pancreatic tumors and 3% of all intraductal pancreatic neoplasms. Reports on intraductal tubulopapillary neoplasm have been rare, and most have described it in the classic macroscopic presentation. We describe a unique case of intraductal tubulopapillary neoplasm wherein the pancreatic duct has completely expanded to fill the borders of the pancreas without any noticeable remaining parenchyma. Global pancreatic parenchymal atrophy has occurred in this patient due to the marked distension of the intraductal tubulopapillary neoplasm. The extent of intraductal expansion makes recognition of commonly observed radiological signs difficult, as there is no clear demarcation between intraductal tumor growth and dilated duct without tumor growth. As more is understood about intraductal tubulopapillary neoplasms, it will be important to be able to recognize the classic and unique radiological presentations in order to provide prompt diagnosis and treatment for these patients.