EBV相关性淋巴上皮瘤样肝内胆管细胞癌的临床病理特征

Clinicopathologic features of Epstein-Barr virus associated lymphoepithelioma-like intrahepatic cholangiocarcinoma

目的探讨EBV相关性淋巴上皮瘤样肝内胆管细胞癌的临床病理学特点。方法通过HE、免疫组化染色、EBER原位杂交检测,对3例EBV相关性淋巴上皮瘤样肝内胆管细胞癌进行形态学观察,并分析其临床病理特征。结果2例男性,1例女性。年龄43~67岁,平均年龄57.3岁,肿瘤大部分由分化好的腺癌组成,伴间质显著增生;其中一例伴有未分化的癌,瘤细胞界限不清,呈合体状,瘤细胞胞质丰富,核呈空泡状,核仁明显,核分裂易见。两种肿瘤成分间质内均可见大量淋巴细胞及浆细胞浸润,并浸润肿瘤细胞间。免疫组化:CK7、CK19及Villin(+),CK20、CDX2、Hepatocyte、Arginase-1、AFP、Glypican-3及CD34(-),Ki-67约20%~30%(+);原位杂交检测EBER(+)。结论EBV相关性淋巴上皮瘤样肝内胆管细胞癌是一种少见的胆管癌亚型,其形态学独具特征,确诊需要结合形态学和免疫组化检测及EBER原位杂交检测。

Objective To investigate the features of EBV associated lymphoepithelioma-like intrahepatic cholangiocarcinoma.Methods Three cases of lymphoepithelioma-like intrahepatic cholangiocarcinoma were studied by light microscopy,immunohistochemical staining and EBV detected by in situ hybridization for Epstein-Barr virus(EBV)-encoded RNA(EBER).Results The tumors were composed of two different components:one was composed of welldifferentiated adenocarcinoma with marked interstitial hyperplasia,and the other was undifferentiated carcinoma.The tumor cells were polygonal with a central nucleus containing prominent nucleoli.The cytoplasm was abandunt with indistinct cell borders,resulting in a syncytial appearance.Mitotic figures were easy to be seen.A large number of lymphocytes and plasma cells infiltrated in the stroma of both components and infiltrated between tumor cells.Immunohistochemical staining showed that the tumor cells were positive for CK7,CK19 and villin,but negative for CK20,CDX2,Hepatocyte,arginase-1,AFP,glypican-3 and CD34;Ki-67 proliferative index was about 20%~30%.In situ hybridization for EBER showed positive nuclear signal in tumor cells.Conclusion EBV associated lymphoepithelioma-like intrahepatic cholangiocarcinoma is a rare subtype of cholangiocarcinoma with special morphology,and its diagnosis mainly depends on histopathology,immunohistochemistry and in situ hybridization for EBER.