Primary liver carcinoma exhibiting dual hepatocellular-biliary epithelial differentiations associated with citrin deficiency - A case report
Primary liver carcinoma exhibiting dual hepatocellular-biliary epithelial differentiations associated with citrin deficiency - A case report
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DOI：
10.1097/01.mcg.0000225683.29841.9c
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发表时间：
2008-08-01
影响因子：
2.9
通讯作者：
Miyagawa, Shin-ichi
中科院分区：
文献类型：
--
作者：
Soeda, Junpei;Yazaki, Masahide;Miyagawa, Shin-ichi
我们报告了一名50岁男性原发性肝癌患者，表现出与瓜氨酸缺乏症（无症状成人发作II型瓜氨酸血症，CTLN 2）相关的肝细胞和胆管上皮双重分化。虽然到目前为止，有14例CTLN 2肝细胞癌患者的报告，这份报告描述了一个独特的情况下，肝癌显示的特点，肝细胞癌和胆管细胞癌。除了先前报道的14例患者的临床数据外，我们患者的研究结果表明，Citrin缺乏可能是导致肝细胞癌的关键疾病之一，特别是在年轻人中，并且在肝祖细胞的肝癌发生中也起重要作用，肝祖细胞具有分化为肝细胞和胆管细胞的双潜能。
We report a 50-year-old male patient with primary liver carcinoma exhibiting dual hepatocellular and biliary epithelial differentiations associated with citrin deficiency (asymptomatic adult-onset type II citrullinemia, CTLN2). Although so far 14 CTLN2 patients with hepatocellular carcinoma have been reported, this report describes a unique case of liver carcinoma showing the features of both hepatocellular and cholangiocellular carcinoma. In addition to the clinical data of the 14 patients reported previously, the findings in our patient suggest that the citrin deficiency might be one of the key disorders causing hepatocellular carcinoma especially at younger ages and can also play an important role in hepatocarcinogenesis of the hepatic progenitor cells, which have the bipotential to differentiate into both hepatocytes and cholangiocytes.