Intrahepatic cholangiocarcinoma with multicystic, mucinous appearance and oncocytic change

A case is reported herein of intrahepatic cholangiocarcinoma (ICC) with multicystic, mucinous appearance and oncocytic change. Because of liver dysfunction, a 73-year-old woman was hospitalized in early October 2003. She was diagnosed as having ICC of the right hepatic lobe with occlusion of the hilar and perihilar bile ducts by imaging examination. Extended right lobectomy was performed but the patient died of liver failure on the next day. In surgically resected specimens, the tumor (3 x 3 cm) was mainly located in the right lobe, and tumors infiltrated along the biliary tree as well as invading into the adjacent hepatic parenchyma. The tumor mass had a sponge or honeycomb appearance. Microscopically, these tumors were composed of multiple microcysts filled by abundant mucin and lined by micropapillary adenocarcinoma cells. Their cytoplasm was acidophilic, appearing as an oncocyte, and carcinoma cells were positive for mitochondrial antigen in addition to biliary cytokeratins. There were no ovarian-like stromas around these cystic tumors, and communication of the biliary lumen with these carcinomatous cysts was not evident, thus different from biliary mucinous cystadenocarcinoma and intraductal papillary neoplasm of the liver. This is the third case of multicystic mucinous ICC and the present case might have been derived from intrahepatic peribiliary glands.