原发性肺淋巴上皮癌13例临床病理学分析

Clinicopathological analysis of 13 cases with primary pulmonary lymphoepithelioma carcinoma

目的探讨原发性肺淋巴上皮癌的临床病理学特征、影像学特征、免疫表型、PD-L1检测、EBER原位杂交检测及预后。方法回顾性分析2013-2021年间南京市胸科医院13例肺原发性淋巴上皮癌(PPLEC)的临床资料及病理学特征,并进行相关文献复习。结果13例原发性肺淋巴上皮癌患者,年龄45~76岁,男性5例,女性8例。CT表现为肿块影或软组织影,未见空气支气管征及空洞。病理组织学示低分化肿瘤细胞呈合胞体样巢片状分布伴有多量淋巴细胞浸润、间质纤维化及多核巨细胞反应。免疫组织化学示肿瘤细胞CKpan,CK5/6,P40,P63阳性,CK7,TTF-1,NapsinA阴性,淋巴细胞CD20,CD3,CD8阳性,低表达或不表达CD4;Ki67指数较高,多在30%~80%之间;PD-L1检测示8例(61.5%)阳性;EBER原位杂交检测12例(92.3%)阳性。7例血液肿瘤标志物升高(其中5例为鳞癌标志物CYFRA21-1)。本组病例总体预后良好,其中11例术后行放化疗。结论肺原发性淋巴上皮癌是肺癌中非常少见类型,临床及影像学无特异性,确诊主要依靠组织病理学形态,免疫组织化学及EBER原位杂交检测。

Objective To investigate the clinicopathological,imaging,immunophenotype,PD-L1 detection,EBER in situ hybridization and prognosis of primary pulmonary lymphoepithelioma carcinoma.Methods The clinical data and pathological characteristics of 13 cases of primary pulmonary lymphoepithelioma carcinoma(PPLEC)in Nanjing Chest Hospital from 2013 to 2021 were analyzed retrospectively,and the related literature was reviewed as well.Results The age of 13 patients with PPLEC was from 45 to 66 years old,5 male and 8 female.CT showed mass shadow or soft tissue shadow,without air bronchogram sign or cavity.Pathological histology indicated that the poorly differentiated tumor cells were distributed in syncytial honeycomb-like sheet shape,accompanied by a large amount of lymphocyte infiltration,interstitial fibrosis and multinuclear giant cell reaction.Immunohistochemistry showed that tumor cells were CKpan,CK5/6,P40,P63 positive,CK7,TTF-1,NapsinA negative,lymphocytes CD20,CD3,CD8 positive,low expression or no expression of CD4.Ki67 index was high,mostly between 30%and 80%.PD-L1 test showed that 8 cases(61.5%)were positive.EBER in situ hybridization was positive in 12 cases(92.3%).Blood tumor markers were elevated in 7 cases(including 5 cases of squamous cell carcinoma marker CYFRA21-1).The overall prognosis of this group of patients was good,including 11 cases of postoperative radiotherapy and chemotherapy.Conclusion PPLEC is a rare type of lung cancer,with no specificity in clinical and imaging science.The diagnosis mainly depends on histopathological morphology,immunohistochemistry and EBER in situ hybridization.