Abstract
A 64-year-old woman who was pointed out impaired hepatic function at a medical checkup was seen at the hospital. Abdominal ultrasonography and CT revealed a multilocular cystic tumor 3.5cm in size involving solid component at the porta hepatis. With ERC the right hepatic duct did not visualized, and dilated right intrahepatic bile duct and compressed and extended left hepatic duct were shown. Abdominal angiography revealed a compressed and extended caudate lobe branch. From these imaging findings, a most probable diagnosis of cystic adenocarcinoma positioned in the porta hepatis was made, and the patient was operated on. Operative procedures included percutaneous transhepatic portal embolization followed by extended excision of the right lobe, excision of the bile duct, and left Roux-Y hepatocholangiojejunostomy. The tumor had enrolled the right hepatic duct, and the major part of the tumor existed in the bile duct extramurally covering from the confluence of the right and left hepatic ducts to right intrahepatic first branch, though a part of it protruded into the right hepatic ductal space. Histopathologically it was well differentiated papillary adenocarcinoma limiting in the cyst.
Cystic adenocarcinomas positioned in the porta hepatis are so rare that four cases in addition to this case have been reported so far. Since the right and left hepatic ducts are adjacent to the hepatic parenchyma and connect to the intrahepatic bile duct, the primary site can not be determined in not a few cases. In the treatment of the disease, hepatectomy with a sufficient distance from the tumor, excision of the hepatic duct and lymph nodes dissection are neceessary.
