Intraductal Papillary Neoplasm of the Bile Duct: Case Series

Abstract

The intraductal papillary neoplasm of the bile duct (IPNB) traditionally called “biliary papillomatosis” is a extremely rare entity, regarded as the biliary equivalent of intraductal papillary mucinous neoplasm of the pancreas. It is considered a premalignant lesion, which progresses in a multistep fashion towards invasive cholangiocarcinoma. IPNB can occur in any segment of the bile duct. The most common radiological findings are dilatation of the bile duct and the presence of intraductal masses. Free margin resection is the treatment of choice for IPNB but could require extreme HPB surgery including liver transplantation. Published papers about IPNB are usually from Asia. Methods: Retrospective study 2014-2020 made in two HPB Units. We checked our prospective database looking for IPNB. We studied patient data, diagnostic methods, surgical procedures performed, pathologic findings and follow-up. Results: We found three cases. After several diagnostic methods, we found intraductal biliary mass and dilatation of the bile duct Results are in Table I.EP229_Table ICaseAge/GenderMain SymptomDiagnostic Methods (DM)LocationMultifocal/UniqueType of SurgeryClavien168 FemaleJaundiceUS,CT,CPRE, SPYGLASSExtrahepatic BDMultifocalBD resection + HJIIIA262 FemaleAbdominal painUS,CT,USE,MRIExtrahepatic BDUniqueBD resection + HJII373 MaleJaundiceUS,CT,USEExtrahepatic BD+ PancreasMultifocalPancreatoduodenectomyIISize Big TumorTNM/RFinal HistologyFollow-Up (M)RelapseDisease Free Survival (M)Overall Survival11,5 CMT1N0M0R1Colangiocarcinoma on IPNB Pancreatobiliary4NO4422 CM-R0IPNB Gastric44NO444432,5 CMT2N2M0R1Colangiocarcinoma on IPNB Pancreatobiliary14SI914 Open table in a new tab Conclusions: IPNB is an extremely rare disease. Diagnosis is not easy and many diagnostic methods are needed to obtain a correct preoperative diagnosis. Extrahepatic bile duct was the most frequent location. In two patients, cholangiocarcinoma was present in surgical specimen, one of them with lymph node spread who implied short survival. Multifocal and unique mass cases are two very different scenarios of the same entity. A European retrospective multicentric study could give information about incidence and characteristics of European patients with IPNB.