A CHILDHOOD FIBROLAMELLAR HEPATOCELLULAR-CARCINOMA WITH INCREASED AROMATASE-ACTIVITY AND A NEAR TRIPLOID KARYOTYPE

We report a 15-year-old boy with hepatocellular carcinoma (HCC) of the fibrolamellar type. He presented with advanced disease and a non-rese ctable tumor. Clinical features included marked gynecomastia which had been present for 3 years, failure to enter puberty, and failure to th rive. These features might have been due to a high aromatase activity of the tumor. The course of the illness suggested that the tumor had b een present for at least 3 years prior to diagnosis. At diagnosis the patient had multiple metastases which included infiltrated ascites. Cy togenetic analysis of the ascites revealed a near triploid karyotype w ith 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. (C) 1997 Wiley-Liss, Inc.