Biliary Lesion? Might Want To Consider An Ipmn

Intraductal papillary mucinous neoplasms (IPMNs) are mucin-producing papillary neoplasms of the pancreatic or biliary ductal system that exhibit cellular dysplasia, produce mucin and as a result lead to ductal obstruction. There are few cases of IPMN's arising from the biliary tree, many of which are reported in East Asian populations. IPMN of the biliary tract has a higher propensity to undergo malignant transformation when compared to its pancreatic counterpart. Cholangioscopy has been proposed as a preferred tool in diagnosing Intraductal papillary mucinous neoplasms of the biliary tract (IPMN-BT) given the ability for direct endoscopic visualization. In our case, a 64-year-old male with hypertension and hyperlipidemia presented with jaundice, diarrhea and weight loss. The patient was found to have a cholestatic liver injury with a bilirubin of 12.7 and alkaline phosphatase of 341 with mild elevations in AST and ALT. An abdominal ultrasound revealed an extrahepatic intraductal mass lesion. In addition to this, an MRI of the abdomen revealed a T1 isointense, T2 hypointense polypoid soft tissue mass (1.9cm x 1.0cm) within the hilar common hepatic duct. The patient underwent ERCP with placement of a common bile duct stent and was discharged home but represented ten days later with similar symptoms and labs. ERCP was re-performed with cholangioscopy and the common hepatic duct stent was removed, as the stent was filled with mucous requiring significant lavage. Subsequently, Cholangioscopy was used to image the biliary tract, biopsies were taken revealing IPMN-BT. The patient then underwent a right hepatectomy with extrahepatic bile duct resection, with Roux-en-Y hepaticojejunostomy. Our case illustrates how per oral cholangioscopy is a helpful tool to aid in the diagnosis of IPMN's of the biliary tract.1393_A.tif Figure 1: A. Ultrasound showing exophytic extrahepatic intraductal mass-like lesion. B. Ultrasound with Doppler re-demonstrating intraductal mass. C. MRI abdomen showing mildly enhancing 1.9cm x 1.0cm polypoid soft tissue mass within the common hepatic duct.1393_B.tif Figure 2: Endoscopic Retrograde Cholangiopancreatography showing dilated intrahepatic and extraheaptic biliary ducts with altered signal intensity lesions at the biliary bifurcation of left and right hepatic duct.1393_C.tif Figure 3: A. Intraductal papillary neoplasm of the bile duct with hematoxylin and eosin staining. 10x magnification. B. 20x magnification demonstrates papillary structures lined by predominately low grade intestinal type mucinous epithelium consistent with IPMN-BT.