LAUSR

Abstract Introduction: Intraductal papillary neoplasms of the bile duct (IPNB) is a kind of rare disorder with low incidence but high misdiagnosis due to untypical symptoms and non-specific laboratory indicators. Herein, we report a case of cystic type IPNB with infiltrating carcinoma of the intrahepatic bile duct presented as a single giant cystic mass of the liver. Patient concerns: A 51-year-old woman was admitted due to right upper abdominal discomfort for 10 months. Physical examination indicated no specific finding. Laboratory tests showed that serum total bilirubin and carcinoembryonic antigen level was mildly elevated. Ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) of abdomen indicated a giant lobulated cystic lesion involving the left, right and the caudate lobes of liver. There were multiple small nodules of different sizes with papillary or coral reef-like pattern protruding into the cystic lumen from the inner wall. Diagnosis: The patient was diagnosed as malignant tumors of intrahepatic bile duct. Interventions: She received radical resection of the lesion by hepatectomy. Outcomes: The postoperative pathological examination revealed an IPNB with infiltrating carcinoma of the intrahepatic bile duct. This patient had an uneventful postoperative recovery and was discharged on day 21 post-surgery. Until 35 months after surgery, there is no recurrence or metastasis. Conclusion: The CT and MRI can show certain morphologic features including the segmental cystic dilatation of intrahepatic bile ducts and the pathological details of papillary tumors inside the lesion. Cystic IPNB with infiltrating carcinoma of the intrahepatic bile duct can be treated with surgery.