Abstract
We report a case of clear cell carcinoma of cystic duct in a 78-year-old man, who was referred to our hospital with the complaint of severe obstructive jaundice. Abdominal CT scan showed intrahepatic bile duct dilatation and a papillary tumor in the extrahepatic bile duct and the cystic duct. Since microscopic examination of the biopsy specimen showed poorly differentiated adenocarcinoma, we performed extrahepatic bile duct resection and regional lymph node dissection. Pathological examination revealed that most of the tumor cells had large clear cytoplasm and we diagnosed clear cell carcinoma of cystic duct. Ten months after operation, liver metastases were detected. After chemotherapy (GEM+CDDP), we performed liver resection for metastatic clear cell carcinoma. A case of primary clear cell carcinoma of the cystic duct is extremely rare.