Abstract
Intraductal papillary neoplasm of the liver (IPNB) is characterized by dilated intrahepatic bile ducts filled with a noninvasive papillary or villous biliary neoplasm covering delicate fibrovascular stalks. Dilated bile ducts are fusiform or cystic. One-third of IPNB secrete mucin in the duct lumen (mucin secreting IPNB). IPNB is classified into adenoma, carcinoma-in-situ and invasive carcinoma according to differentiation or into pancreaticobiliary type, intestinal type, gastric type, and oncocytic type according to cytological types, which are considered to be associated with prognosis. IPNB develops predominantly in the left lobe and can be diagnosed using US, CT and MRI. Superficial spread along the bile duct epithelium can be evaluated by choledochoscopy. Although curative resection contributes to good prognosis, re-evaluation of diagnostic criteria, treatment strategy and prognosis is mandatory.