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肝结节性肝母细胞瘤,含肝囊肿、胆管板畸形和肝门周围腺体。

Hepatic nodular hamartoma containing liver cysts, ductal plate malformations and peribiliary glands.

机构信息

Department of Pathology, Shizuoka City Shimizu Hospital, Shizuoka, Japan.

出版信息

Hepatol Res. 2011 Jan;41(1):93-8. doi: 10.1111/j.1872-034X.2010.00746.x.

DOI:10.1111/j.1872-034X.2010.00746.x
PMID:21199202
Abstract

An 83-year-old man with hepatocellular carcinoma was found to have a low-echoic and low-density tumor measuring 7.2 cm × 5.6 cm. Caroli's disease was absent. Clinical diagnosis was intrahepatic cholangiocarcinoma. Three cores of liver biopsy were obtained from the tumor. Histologically, it consisted of liver cysts, ductal plate malformations, peribiliary glands, hepatocytes, portal tracts and mesenchymal tissue. Apparent features of cirrhosis were not found. The liver cysts were lined by a layer of cuboidal cells with multiple papillary protrusions. The ductal plate malformations resembled fetal ductal plates. The peribiliary glands were seromucous glands. Immunohistochemically, these abnormal ductal structures showed positive reaction to biliary type cytokeratins, namely, cytokeratin (CK)7, CK8, CK18 and CK19. Mucin gene expression showed that these biliary structures are positive for fetal antigen MUC1. MUC6 is also positive in them. Aberrant expression of CD10 was observed in these biliary structures. MUC2, MUC5AC and CDX2 were negative. The author thinks that this lesion is hepatic hamartoma with ductal plate malformations, liver cysts and peribiliary glands. To the best of the author's knowledge, this type of liver nodule has not been reported in the published work.

摘要

一位 83 岁男性,患有肝细胞癌,发现一个低回声和低密度的肿瘤,大小为 7.2×5.6cm。不存在 Caroli 病。临床诊断为肝内胆管细胞癌。从肿瘤中获得了 3 个肝活检核心。组织学上,它由肝囊肿、胆管板畸形、胆管周围腺体、肝细胞、门管区和间充质组织组成。未发现明显的肝硬化特征。肝囊肿由一层具有多个乳头状突起的立方细胞衬里。胆管板畸形类似于胎儿胆管板。胆管周围腺体为浆液黏液腺。免疫组织化学染色显示,这些异常的胆管结构对胆管型细胞角蛋白呈阳性反应,即细胞角蛋白(CK)7、CK8、CK18 和 CK19。粘蛋白基因表达显示这些胆管结构对胎儿抗原 MUC1 呈阳性。它们也对 MUC6 呈阳性。这些胆管结构中观察到 CD10 的异常表达。MUC2、MUC5AC 和 CDX2 为阴性。作者认为该病变为肝错构瘤伴胆管板畸形、肝囊肿和胆管周围腺体。据作者所知,这种类型的肝结节尚未在已发表的文献中报道。

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