title: Liver tumors in children with metabolic disorders.
abstract: Hepatic neoplasia is a rare but serious complication of metabolic diseases in children. The risk of developing neoplasia, the age at onset, and the measures to prevent it differ in the various diseases. We review the most common metabolic disorders that are associated with a heightened risk of developing hepatocellular neoplasms, with a special emphasis on reviewing recent advances in the molecular pathogenesis of the disorders and pre-clinical therapeutic options. The cellular and genetic pathways driving carcinogenesis are poorly understood, but best understood in tyrosinemia.
authors.affiliation: Department of Pathology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX 77030, USA.
authors.forename: Deborah A
authors.initials: DA
authors.lastname: Schady
authors.affiliation: Department of Pathology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX 77030, USA.
authors.forename: Angshumoy
authors.initials: A
authors.lastname: Roy
authors.affiliation: Department of Pathology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX 77030, USA.
authors.forename: Milton J
authors.initials: MJ
authors.lastname: Finegold
keywords: Cirrhosis; hemochromatosis; tyrosinemia; α-1 antitrypsin (AAT)
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