Hepatoblastoma in Alagille Syndrome

Abstract

Background and Aim: Alagille syndrome (AGS) is a rare multisystem genetic disorder of autosomal dominant inheritance classically characterized by intrahepatic bile duct paucity in conjunction with various combinations of extrahepatic manifestations and characteristic facial features. Apart from various hepatic manifestations these patients are also at risk of developing a wide variety of liver tumours including nodular hyperplasia of the liver, adenomas and hepatocellular carcinomas. Although there are reports of numerous cases of hepatocellular carcinoma among children with AGS but there is a dearth of cases of hepatoblastoma in young children.