A Case Report of Biliary Atresia Associated With Hepatocellular Carcinoma

Abstract

The combination of congenital biliary atresia (CBA) and hepatocellular carcinoma (HCC) is rare. However with the increasing number of patients with CBA, the significance of its assosiation with HCC is becoming more important. This paper presents a case of CBA associated with HCC. The patient was a 16 years-old girl. She was opeated on in our hospial 140 days of age after having been diagnosed as CBA (the type of I cyst) . Intraoperative cholangiography showed that development of intrahepatic bile ducts were cosiderable and the wall of the left hepatic bile duct was irregular. Histological examination of the liver revealed biliary fibrosis. After the operation she showed no further symptoms until she complained of fever, jaundice and epigastric pain at 16 years old. Laboratory data revealed high levels of AFP (> 50,000ng/ml) , CEA (14ng/ml). HBsAg was negative. A 7cm hypovascular mass was detected in the lateral segment of the liver using CT scan, echograpy and angiography. Needle biopsy was carried out and a well differantiated HCC was diagnosed. 5'FUDR was deliverd but she died 5 months after the onset of the symptom. This was thought to be the first case where a patient has been free of jaundice until association with HCC. This case would show that it is important to cosider the association with HCC in the follow-up period after an operation for CBA.