Abstract
Biliary atresia (BA) is a rare cholangiopathy in neonates, leading to end-stage liver failure in the first years of life, when left untreated. Kasai procedure aims to restore biliary drainage to achieve native liver survival. While liver transplantation is the necessary treatment in children with failure of the Kasai procedure, the number of native liver survivors in the long-term remains around 20-30%. Reports on malignancies in native livers of children, adolescents and adults with BA are increasing, including cases of hepatocellular carcinoma, cholangiocarcinoma, hepatoblastoma and small intestinal adenocarcinoma. In this review we summarize the reports, with focus on tumor markers, imaging technologies and reported outcomes. Furthermore, we discuss recent advances in surveillance strategies in adults with chronic liver impairment.