Intraductal tubulopapillary neoplasms of the pancreas and biliary tract: The black swan of hepatobiliary surgery

Intraductal tubulopapillary neoplasms (ITPNs) of the pancreas and biliary tract are rare pre-malignant entities of the biliary tract and pancreas that are difficult to diagnose preoperatively. While there are imaging characteristics that can differentiate these lesions from more common entities like adenocarcinoma or intraductal papillary mucinous neoplasms (IPMN), ITPNs are not always distinctive. Herein we present two cases of ITPN, one of biliary and the other of pancreatic origin, which had a preoperative diagnosis of cholangiocarcinoma and IPMN, respectively. We discuss our findings in these cases, patient presentation and course, review the radiographic and pathologic findings, and propose a more effective approach to the preoperative workup and diagnosis of ITPN based on our review of the contemporary literature.