Hany M A, Betts D R, Schmugge M, Schönle E, Niggli F K, Zachmann M, Plüss H J
Department of Oncology, University Children's Hospital, Zürich, Switzerland.
Med Pediatr Oncol. 1997 Feb;28(2):136-8. doi: 10.1002/(sici)1096-911x(199702)28:2<136::aid-mpo8>3.0.co;2-l.
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.
我们报告一名15岁患纤维板层型肝细胞癌（HCC）的男孩。他就诊时已处于疾病晚期，肿瘤无法切除。临床特征包括已存在3年的明显男性乳腺增生、青春期发育延迟和生长发育迟缓。这些特征可能归因于肿瘤的高芳香化酶活性。病程表明，肿瘤在诊断前至少已存在3年。诊断时患者已有多处转移，包括浸润性腹水。腹水的细胞遗传学分析显示接近三倍体核型，存在细胞间差异以及1号染色体长臂异常。据我们所知，这是儿童纤维板层型肝癌的首例核型报告。
