title: A childhood fibrolamellar hepatocellular carcinoma with increased aromatase activity and a near triploid karyotype.
abstract: We report a 15-year-old boy with hepatocellular carcinoma (HCC) of the fibrolamellar type. He presented with advanced disease and a non-resectable tumor. Clinical features included marked gynecomastia which had been present for 3 years, failure to enter puberty, and failure to thrive. These features might have been due to a high aromatase activity of the tumor. The course of the illness suggested that the tumor had been present for at least 3 years prior to diagnosis. At diagnosis the patient had multiple metastases which included infiltrated ascites. Cytogenetic analysis of the ascites revealed a near triploid karyotype with cell-to-cell variation and an abnormality of chromosome 1 q. This to our knowledge is the first karyotype report of fibrolamellar HCC in a child.
authors.affiliation: Department of Oncology, University Children's Hospital, Zürich, Switzerland.
authors.forename: M A
authors.initials: MA
authors.lastname: Hany
authors.forename: D R
authors.initials: DR
authors.lastname: Betts
authors.forename: M
authors.initials: M
authors.lastname: Schmugge
authors.forename: E
authors.initials: E
authors.lastname: Schönle
authors.forename: F K
authors.initials: FK
authors.lastname: Niggli
authors.forename: M
authors.initials: M
authors.lastname: Zachmann
authors.forename: H J
authors.initials: HJ
authors.lastname: Plüss
mesh_terms: D000293:Adolescent; D001141:Aromatase / Q000378:metabolism*; D006528:Carcinoma, Hepatocellular / Q000201:enzymology* / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007621:Karyotyping; D008113:Liver Neoplasms / Q000201:enzymology* / Q000235:genetics* / Q000473:pathology; D008297:Male; D009363:Neoplasm Proteins / Q000378:metabolism*; D011123:Polyploidy
chemical_list: D009363:Neoplasm Proteins; D001141:Aromatase