原发鼻咽部EB病毒阳性大细胞神经内分泌癌2例临床病理学分析  

Primary Epstein-Barr virus-positive large cell neuroendocrine carcinoma of nasopharynx:a clinicopathological analysis of two cases

作　　者：庾建中 高敏[1] 袁泳锨 徐咏强 易慕华 YU Jian-zhong;GAO Min;YUAN Yong-xian;XU Yong-qiang;YI Mu-hua(Department of Pathology,the Tenth Affiliated Hospital,Southern Medical University/Dongguan Pepole s Hospital,Dongguan Key Laboratory of Clinical Pathology,Dongguan 523059,China)

机构地区：[1]南方医科大学第十附属医院/东莞市人民医院病理科,东莞市临床病理重点实验室,广东东莞523059

出　　处：《诊断病理学杂志》2025年第1期13-17,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨原发鼻咽部EB病毒阳性大细胞神经内分泌癌(EBV+LCNEC)的临床病理特征。方法分析2例原发鼻咽部EBV+LCNEC的临床病理特征,并系统性复习相关文献。结果2例病例头颅MRI均显示受累鼻咽部黏膜增厚,T1WI为低信号,T2WI为高信号,周围淋巴结可见融合。镜下见瘤细胞呈弥漫巢片状或条索状分布,细胞中等偏大,胞质中等量、嗜酸;核染色质较细腻,核仁明显,核分裂象多见(约10个/HPF)。免疫组化表达CKpan、CD56、INSM1、Syn、EMA,Ki-67增殖指数约90%~95%,2例行EBER原位杂交检测均显示阳性。结论原发鼻咽部EBV+LCNEC是一种非常罕见的头颈部上皮性神经内分泌肿瘤,形态学上与肺的大细胞神经内分泌癌类似,因此必须结合组织学形态、免疫组织化学标记进行诊断和鉴别诊断,并行EBER原位杂交检测。Objective To investigate the clinicopathological characteristics of primary nasopharyngeal Epstein Barr virus positive large cell neuroendocrine carcinoma(EBV+LCNEC).Methods The clinical and pathological characteristics were analyzed in 2 cases of primary nasopharyngeal EBV+LCNEC,and relevant literature was systematically reviewed.Results Two cases of head MRI showed thickening of the affected nasopharyngeal mucosa,with low signal on T1WI and high signal on T2WI,and fusion of surrounding lymph nodes.Histologically,the tumor cells were distributed in a diffuse nest-like or cord-like pattern,with medium to large cells and an equal amount of acidophilic cytoplasm;The nuclear chromatin was delicate,the nucleolus was obvioused,and nuclear mitotic figures were commonly noted(about 10/HPF).Immunohistochemistry showed tumor cells expressed CKpan,CD56,INSM1,Syn(weakly positive),and Ki-67 proliferation index was approximately 90%-95%.Two routine EBER in situ hybridization tests showed positive results.Conclusion Primary nasopharyngeal EBV+LCNEC is a very rare epithelial neuroendocrine tumor of the head and neck,morphologically similar to large cell neuroendocrine carcinoma of the lungs.Therefore,it is necessary to combine histological morphology,immunohistochemical markers for diagnosis and differential diagnosis,and perform EBER in situ hybridization detection.

关 键 词：大细胞神经内分泌癌 EB病毒 鼻咽癌 临床病理学特征 免疫组化 

分 类 号：R739.63[医药卫生—肿瘤]