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具有中间型(肝细胞-胆管细胞)表型的原发性肝肿瘤:一种祖细胞肿瘤?

Primary liver tumour of intermediate (hepatocyte-bile duct cell) phenotype: a progenitor cell tumour?

作者信息

Robrechts C, De Vos R, Van den Heuvel M, Van Cutsem E, Van Damme B, Desmet V, Roskams T

机构信息

Department of Pathology, University of Leuven, Belgium.

出版信息

Liver. 1998 Aug;18(4):288-93. doi: 10.1111/j.1600-0676.1998.tb00168.x.

Abstract

A 57-year-old female patient presented with painless obstructive jaundice and mild mesogastric pain; she was in good general condition on admission. Abdominal ultrasonography revealed diffuse tumoral invasion of the liver, suggesting diffuse metastases. A liver biopsy showed a tumour with a trabecular growth pattern, composed of uniform relatively small cells, very suggestive of an endocrine carcinoma. Additional immunohistochemical stains, however, did not show any endocrine differentiation, but showed positivity for both hepatocyte-type cytokeratins (cytokeratin 8 and 18) and bile duct-type cytokeratins (cytokeratin 7 and 19). In addition, parathyroid hormone-related peptide, shown to be a good marker for cholangiocarcinoma, was immunoreactive. Electron microscopy revealed tumour cells with an intermediate phenotype: the cells clearly showed hepatocyte features on one hand and bile duct cell features on the other hand. Nine days after admission, the patient died due to liver failure and hepatic encephalopathy. Autopsy excluded another primary tumour site. Overall, this tumour was a primary liver tumour with an intermediate phenotype and with a very rapid clinical course. The intermediate (between hepatocyte and bile duct cell) phenotype suggests an immature progenitor cell origin, which is concordant with a rapid clinical course. This type of tumour has not been described previously and provides additional evidence for the existence of progenitor cells in human liver.

摘要

一名57岁女性患者表现为无痛性梗阻性黄疸和轻度上腹部疼痛;入院时全身状况良好。腹部超声检查显示肝脏弥漫性肿瘤浸润,提示弥漫性转移。肝脏活检显示肿瘤呈小梁状生长模式,由相对较小的均匀细胞组成,高度提示为内分泌癌。然而,进一步的免疫组化染色未显示任何内分泌分化,但对肝细胞型细胞角蛋白(细胞角蛋白8和18)和胆管型细胞角蛋白(细胞角蛋白7和19)均呈阳性。此外,甲状旁腺激素相关肽(已证明是胆管癌的良好标志物)呈免疫反应性。电子显微镜检查显示肿瘤细胞具有中间表型:细胞一方面明显显示肝细胞特征,另一方面显示胆管细胞特征。入院九天后,患者因肝衰竭和肝性脑病死亡。尸检排除了其他原发肿瘤部位。总体而言,该肿瘤是一种具有中间表型且临床病程非常迅速的原发性肝肿瘤。这种中间(肝细胞和胆管细胞之间)表型提示其起源于未成熟的祖细胞,这与快速的临床病程一致。这种类型的肿瘤此前尚未见报道,为人类肝脏中祖细胞的存在提供了额外证据。

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