DIAGNOSTIC PROBLEMS IN HEPATOLOGY	Semin Liver Dis 2007; 27: 427-431 DOI: 10.1055/s-2007-991518
© Thieme Medical Publishers
Hepatocellular Carcinoma and Congenital Absence of the Portal Vein in a Child Receiving Growth Hormone Therapy for Turner Syndrome
Raffaella A. Morotti1,3, Mary Killackey2, Benjamin L. Shneider2,3, Anthony Repucci4, Sukru Emre2,3, Swan N. Thung1
1 Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine, New York, New York 2 Recanati Miller Transplantation Institute, Mount Sinai School of Medicine, New York, New York 3 Department of Pediatrics, Mount Sinai School of Medicine, New York, New York 4 Connecticut Children's Medical Center, Hartford, Connecticut

ABSTRACT

Turner syndrome patients are reported to have no increase in the relative risk of cancer, and hepatocellular carcinoma in particular has never been reported in this syndrome. However, Turner syndrome patients are known to have an increased prevalence of liver lesions, some of which, like focal nodular hyperplasia, are related to vascular anomalies. We report the case of a young patient with Turner syndrome, treated with recombinant human growth hormone, who was found to have absence of the portal vein, focal nodular hyperplasia, and hepatic adenoma and who developed a large hepatocellular carcinoma requiring liver transplant.

KEYWORDS

Turner syndrome - absence of portal vein - focal nodular hyperplasia - hepatocellular carcinoma