ABSTRACT
Biliary Tract Intraductal Papillary Mucinous Neoplasm (BT-IPMN) is a very rare entity, gradually emerging into attention as sporadic cases are being reported worldwide. In this brief report we discuss about such an entity from our part of the world, based on a case from our institution. A 47-year-old female was referred to our department with jaundice, intermittent fever with chills and rigor of 6 weeks duration. Initial evaluation revealed obstructive jaundice with distended gall bladder. Imaging with ultrasonogram (USG) and magnetic resonance imaging (MRI) showed hugely dilated intra and extrahepatic biliary radicles with multiple and diffuse soft tissue lesions fi lling the common bile duct (CBD) extending to the ductal system of left lobe of liver. A side viewing endoscopy demonstrated mucin extruding from a prominent ampulla of Vater. The patient was managed successfully by left hepatectomy with pancreaticoduodenectomy (HPD). Gross pathological examination of the specimen showed marked dilatation of intra and extra hepatic bile ducts with multiple polypoidal lesions and plenty of mucin fi lling the entire biliary ductal system. Histopathology revealed predominantly intraductal papillary mucinous adenocarcinoma at the hilum extending to left bile duct with diffuse dysplastic changes throughout the biliary tree. Thus the clinical, radiological and pathological features of this lesion clearly fi t into the diagnosis of BT-IPMN, which is slowly being established as a defi nite clinical entity with features much similar to its pancreatic counterpart.