Malignant intraductal papillary neoplasm of the bile duct with intrahepatic rupture

Abstract

Mucin producing intraductal papillary neoplasm of the bile duct(IPMN-B) has previously been reported in various terms including intraductal papillary neoplasia of the liver, mucin-hypersecreting biliary papillomatosis, and mucin-producing cholagiocarcinoma. Malignant IPMN-B is a referred as intraductal growth type of intrahepatic chlangiocarcinoma (ICC), which is a rare disease and reveal a more favorable prognosis. Therefore we reported malignant intraductal papillary neoplasm of the bile duct with intrahepatic rupture. Case report: A 52-year male was admitted for RUQ pain and fever. Vital sign was stable and he showed mild tenderness of RUQ abdomen. Initial laboratory test revealed WBC 14700, Hg 11.7, Plt 132000, and AST/ALT of 1880/1770IU/L, T B 2.23mg/dL, AFP of 1.47ng/mL, ca19-9 of 16.2 U/mL, and Initial ABDO CT was shown subcapsular hemobilia in right liver with internal enhancing mass in left bile duct, so diagnosed malignant IPMN-B with intrahepatic rupture. Pet-CT was shown no distant metastasis and the high FDG uptake in the tumor with in left bile duct. The S4 bile duct with mass was attached to middle hepatic vein, so we performed a modified left hepatectomy with segmental resection of middle-hepatic vein and reconstruction with gore-tex. Operation time was 7hr 30 min and blood loss was 350cc. It diagnosed well differentiated, 6x4x2.5cm sized, intraductal papillary carcinoma of the bile duct. There was no metastasis of regional lymph node. The patient had no postoperative complications and was discharged. Conclusion: Through precise preoperative diagnosis and wide complete resection of malignant IMN-B, a more favorable prognosis can be expected.