Hepatocarcinoma in a child with the Alagille syndrome

Am J Dis Child. 1987 Jun;141(6):698-700. doi: 10.1001/archpedi.1987.04460060114050.


A child with the Alagille syndrome of intrahepatic bile duct paucity developed hepatocarcinoma. Disabling cirrhosis had rendered this child a suitable candidate for transplantation before the discovery of carcinoma. However, the extension of the tumor outside the liver precluded the performance of this potentially life-saving operation. Serial monitoring of the serum alpha-fetoprotein concentration may be of value in the early identification of tumors in pediatric candidates for transplantation.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / pathology*
  • Bile Ducts, Intrahepatic / abnormalities*
  • Biopsy
  • Carcinoma, Hepatocellular / pathology*
  • Child, Preschool
  • Cholestasis, Intrahepatic / congenital*
  • Cholestasis, Intrahepatic / pathology
  • Female
  • Humans
  • Liver / pathology
  • Liver Cirrhosis / pathology
  • Liver Neoplasms / pathology*
  • Syndrome