原发性肺淋巴上皮瘤样癌的临床特征及预后

Clinical features and outcome of primary pulmonary lymphoepithelioma-like carcinoma

目的探讨原发性肺淋巴上皮瘤样癌的临床特征、治疗及预后。方法回顾性分析2015年1月至2019年12月在复旦大学附属中山医院厦门医院及复旦大学附属中山医院诊治的39例原发性肺淋巴上皮瘤样癌患者的临床资料,并进行总结。结果19例(48.7%)患者体检时发现肿瘤,症状无特异性。83.9%(26/31)患者程序性死亡配体1(PD-L1)阳性,97.2%(35/36)患者EB病毒编码的小RNA(Epstein-Barr virus encoded small RNA,EBER)阳性。基因检测发现1例合并ROS-1基因重排,未检测到EGFR基因突变及ALK基因重排。64.1%(25/39)患者进行手术治疗,无法手术的患者采取多学科综合诊治策略。中位随访33.7(23.4,47.7)个月,中位生存时间未达到。4例患者死亡,其中1例合并ROS-1基因重排,生存期24.4个月;另3例生存期为49.7、25.3、69.7个月。相关性分析显示PD-L1表达情况不是生存时间的独立影响因素。结论原发性肺淋巴上皮瘤样癌是一种罕见类型的肺恶性肿瘤,发生与EB病毒密切相关,PD-L1高表达,肺癌驱动基因突变少见,临床发现时分期常较早,采用以手术为主的多学科综合诊治策略,多数患者预后较好。

Objective To investigate the clinical characteristics,treatment,and prognosis of primary pulmonary lymphoepithelioma-like carcinoma.Methods Clinical data of 39 patients with primary pulmonary lymphoepithelioma-like carcinoma and were hospitalized in Xiamen Branch,Zhongshan Hospital,Fudan University and Zhongshan Hospital,Fudan Universtity from January 2015 to December 2019 were retrospectively analyzed.Results Symptoms were not specific,and 48.7%(19/39)patients were found by physical examination.83.9%(26/31)patients were PD-L1 positive,and 97.2%(35/36)patients were Epstein-Barr virus encoded small RNA(EBER)positive.Genetic testing found that 1 patient had ROS-1 rearrangement,but no EGFR mutation and ALK rearrangement were detected.64.1%(25/39)patients underwent surgical treatment,and the patients who could not be operated were treated with multidisciplinary comprehensive treatment strategy.The median follow-up time was 33.7(23.4,47.7)months,and the median survival time was not reached in all patients.Four patients died,of whom,one patient had a ROS-1 mutation with a survival time of 24.4 months;the survival time of the other 3 patients was 49.7,25.3,69.7months respectively.Correlation analysis showed that the expression of PD-L1 was not an independent risk factor of survival time.Conclusion Primary pulmonary lymphoepithelioma-like carcinoma is a rare type of primary lung cancer.It’s occurrence is closely related to the Epstein-Barr virus.Lung cancer driver gene mutations are rare.A multidisciplinary comprehensive diagnosis and treatment strategy based on surgery is offen adopted.Most patients have a good prognosis.