Intraductal Papillary Neoplasia of Bile Duct: A Rare Liver Tumor

Background Intraductal biliary papillary neoplasia (IPNB) is a rare disease involving both the intrahepatic and the extrahepatic biliary tract. It usually occurs in the 6th and 7th decades of life and may present with acute cholangitis. The pathogenesis, clinical features and outcome are not well documented. Because of the high risk of malignant transformation, surgical resection is the best choice of treatment. Case presentation We present the case of IPNB of the left intrahepatic biliary tract in a 49-year-old male, who was admitted to the hospital with right upper quadrant abdominal pain. Laboratory tests showed mild elevation of C reactive protein (CRP), alanine aminotransferase (ALT), aspartate transaminase (AST) and gamma glutamyl trans-peptidase (GGT). Imaging demonstrated dilatation of the intrahepatic and common bile ducts, and a 2x3 cm mass in the left lobe of the liver. A 3D magnetic resonance cholangiopancreatography (MRCP) confirmed an intraductal polypoid lesion. A typical left hepatectomy was performed. A papillary tumor was found in the left intrahepatic biliary duct. Histolopathological examination demonstrated biliary papillomatosis with high grade dysplasia, but no malignant transformation. No recurrence was detected at 6-month follow-up. Conclusion Intrahepatic biliary papillomatosis is a rare tumor of the biliary ducts, both intrahepatic and extrahepatic. Surgical resection is considered the optimal treatment for biliary papillomatosis because of the risk of malignant transformation. Magnetic resonance cholangiopancreatography is a useful preoperative diagnostic examination that can show papillary lesions in the bile ducts. In patients without distant organ and lymph node metastasis, resection provides long-term survival.