Cystadenocarcinoma of the liver is a rare neoplasm that originates from hepatobiliary epithelium. Primary location of this tumor is generally intrahepatic and most cases are in the right hepatic lobe. Herein we present a case of intrahepatic cystadenocarcinoma in a 53-year-old man who had been followed up for 8 years as hydatic cyst disease of the liver. He was admitted to our hospital with jaundice and loss of appetite. Ultrasound and computed tomography showed a cystic lesion that looked like type II cyst hidatic. Thereafter magnetic resonance imaging revealed a cystic lesion associated with biliary tree. On diagnostic laparotomy peritoneal infiltrations were observed and pathologic examination revealed a biliary cystadenocarcinoma and peritonitis carcinomatosa was diagnosed. Unfortunately correct diagnosis was extremely late and no curative management was possible.
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