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: M A Hany, D R Betts, M Schmugge, E Schönle, F K Niggli, M Zachmann, H J Plüss
• Journal: Medical and pediatric oncology (Med Pediatr Oncol) Vol. 28 Issue 2 Pg. 136-8 (Feb 1997) ISSN: 0098-1532 [Print] United States
• PMID: 8986150 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Neoplasm Proteins
• Aromatase

Topics

• Adolescent
• Aromatase (metabolism)
• Carcinoma, Hepatocellular (enzymology, genetics, pathology)
• Humans
• Karyotyping
• Liver Neoplasms (enzymology, genetics, pathology)
• Male
• Neoplasm Proteins (metabolism)
• Polyploidy