EB病毒相关性淋巴上皮瘤样肝内胆管细胞癌1例报告并文献复习  

EPSTEIN-BARR VIRUS-ASSOCIATED LYMPHOEPITHELIOMA-LIKE INTRAHEPATIC CHOLANGIOCARCINOMA:A CASE REPORT AND LITERATURE REVIEW

作　　者：侯明妤 何冰[1] 卢晓 杨丹丹[1] 郭芷含 李玉军[1] HOU Mingyu;HE Bing;LU Xiao;YANG Dandan;GUO Zhihan;LI Yujun(Department of Pathology,The Affiliated Hospital of Qingdao University,Qingdao 266003,China)

机构地区：[1]青岛大学附属医院病理科,山东青岛266003

出　　处：《精准医学杂志》2023年第2期176-179,共4页Journal of Precision Medicine

基　　金：山东省自然基金资助项目(ZR2017MH009);青岛大学医学+X项目(QDFY+X2021017)。

摘　　要：目的探讨EB病毒相关性淋巴上皮瘤样肝内胆管细胞癌(EBVaLELICC)的病理学特征,以加深临床对该肿瘤的认识。方法收集1例EBVaLELICC患者的临床及病理学资料,对其病理石蜡标本进行免疫组化、原位杂交和基因重排检测,分析该病病理学特点,并复习相关文献。结果患者女,56岁,肿瘤位于肝左外叶,边界清楚,约1.8 cm×1.5 cm×1.2 cm大小。于肝胆外科行腹腔镜下肝左外叶切除术,术后病理学检查示肿瘤细胞呈腺管状,部分不规则、融合或呈筛状,细胞呈合体状,核大、染色质细,可见小核仁,未见核分裂象;间质大量淋巴细胞、浆细胞增生,伴淋巴滤泡形成。免疫组化染色示肿瘤细胞表达CK7、CK19,P53表达率为10%,表现为野生型,Ki-67增殖指数5%,间质淋巴细胞表达CD3、CD4、CD8、CD20,淋巴滤泡生发中心细胞表达BCL-6、CD10,不表达BCL-2,浆细胞表达CD38、Kappa、Lambda,PD-L1(22C3)在肿瘤细胞及间质淋巴细胞均有表达,联合阳性分数值约为30。EBER原位杂交检测示肿瘤细胞弥漫阳性。淋巴细胞免疫球蛋白及T细胞受体基因均呈多克隆重排。患者术前及术后均未接受任何治疗,随访8个月健在,未发现肿瘤复发转移。结论EBVaLELICC是一种罕见的胆管癌亚型,具有独特的病理学特征,患者可从免疫治疗中获益,预后好于普通型胆管细胞癌。Objective To investigate the pathological features of Epstein-Barr virus(EBV)-associated lymphoepithelioma-like intrahepatic cholangiocarcinoma(EBVaLELICC),and to improve the understanding of this disease in clinical practice.Methods Clinical and pathological data were collected from one patient with EBVaLELICC,and immunohistochemistry,in situ hybridization,and gene rearrangement test were performed for the paraffin-embedded tissue sample of this patient.The pathological features of this disease were analyzed,and a literature review was performed.Results The female patient,aged 56 years,had the tumor located in the left lateral lobe of the liver,with a clear boundary and a size of 1.8 cm×1.5 cm×1.2 cm.The patient underwent laparoscopic left lateral segment liver resection in Department of Hepatobiliary Surgery.Postoperative pathology showed that tumor cells had a glandular tubular structure,and some cells had an irregular,fused or sieve-like shape,with large nuclei,fine chromatin,small nucleoli,and no mitotic figures;proliferation of lymphocytes and plasma cells was observed with the formation of lymphoid follicles in the stroma.Immunohistochemical staining showed that CK7,CK19,and P53 had an expression rate of 10%in tumor cells,with wild type and a Ki-67 proliferation index of 5%;stromal lymphocytes showed the expression of CD3,CD4,CD8,and CD20,lymphoid follicular germinal center cells showed the expression of BCL-6 and CD10 and had no expression of BCL-2,and plasma cells showed the expression of CD38,Kappa,and Lambda;PD-L1(22C3)was expressed in both tumor cells and interstitial lymphocytes,with a combined positive score of 30.EBER in situ hybridization assay showed diffuse positivity of tumor cells.Polymerase chain reaction detected multiple clones of lymphocyte immunoglobulin and T cell receptor genes.The patient did not receive any treatment before and after surgery and was alive after 8 months of follow-up,without tumor recurrence or metastasis.Conclusion EBVaLELICC is a rare subtype of cholangiocarcinoma

关 键 词：疱疹病毒4型 人 胆管上皮癌 免疫组织化学 诊断 鉴别 B-淋巴细胞 T-淋巴细胞 

分 类 号：R735.8[医药卫生—肿瘤]