原发性肺淋巴上皮瘤样癌45例临床分析  

Clinical analysis of 45 cases of primary pulmonary lymphoepithelioma-like carcinoma

作　　者：杨栖 王春语 李思儒 左卓[3] 尹存俐 鲁迎春 柳斌[4] YANG Xi;WANG Chunyu;LI Siru;ZUO Zhuo;YIN Cunli;LU Yingchun;LIU Bin(School of Medical and Life Sciences,Chengdu University of Traditional Chinese Medicine,Chengdu,Sichuan 610032,P.R.China;School of Medicine,University of Electronic Science and Technology of China,Chengdu People's Republic of China,Chengdu,Sichuan 610056,P.R.China;Department of Pathology,Sichuan Clinical Research Center for Cancer,Sichuan Cancer Hospital&Institute,Sichuan Cancer Center,Affiliated Cancer Hospital of University of Electronic Science and Technology of China,Chengdu,Sichuan 610042,P.R.China;Department of Medical Oncology,Sichuan Clinical Research Center for Cancer,Sichuan Cancer Hospital&Institute,Sichuan Cancer Center,Affiliated Cancer Hospital of University of Electronic Science and Technology of China,Chengdu,Sichuan 610042,P.R.China)

机构地区：[1]成都中医药大学医学与生命科学学院,四川成都610032 [2]电子科技大学医学院,四川成都610056 [3]四川省肿瘤临床医学研究中心,四川省肿瘤医院·研究所,四川省癌症防治中心,电子科技大学附属肿瘤医院病理科,四川成都610042 [4]四川省肿瘤临床医学研究中心,四川省肿瘤医院·研究所,四川省癌症防治中心,电子科技大学附属肿瘤医院肿瘤内科,四川成都610042

出　　处：《中国呼吸与危重监护杂志》2023年第7期500-505,共6页Chinese Journal of Respiratory and Critical Care Medicine

摘　　要：目的探讨原发性肺淋巴上皮瘤样癌(primary pulmonary lymphoepithelioma-like carcinoma,PPLELC)的临床病理特征、治疗方法以及预后情况。方法回顾性分析四川省肿瘤医院2016年4月—2023年3月收治的PPLELC患者45例,对其临床资料及生存情况进行分析总结。结果45例患者中,28.9%(13/45)患者无症状,97.2%(35/36)患者EB病毒编码的小RNA阳性,83.3%(10/12)患者的程序性死亡配体1(programmed cell death-ligand 1,PD-L1)阳性,肿瘤细胞阳性比例分数波动在10.0%~95.0%。基因检测发现1例表皮生长因子受体基因19-Del突变。45例患者中,28例接受了外科手术治疗,其中接受了辅助化疗者16例,辅助放化疗者8例,接受了新辅助化疗者2例。截至随访截止日期,33例患者存活,12例患者死亡,所有患者均未达到中位生存时间,3年和5年总生存率分别为70.2%和63.2%。28例手术患者的3年和5年无病生存率分别为67.2%和57.6%,13例Ⅳ期患者的无进展生存率分别为58.3%和29.2%。结论PPLELC与EB病毒感染有关,是一种罕见的非小细胞肺癌亚型,大多数患者无表皮生长因子受体或间变性淋巴瘤激酶等常见基因突变,PD-L1阳性表达率高。该病通常采用以手术为主的多方法综合治疗,对放化疗较敏感,预后较其他类型的非小细胞肺癌好。Objective To investigate the clinicopathological features,treatment and prognosis of primary pulmonary lymphoepithelioma-like carcinoma(PPLELC).Methods Forty-five patients with PPLELC admitted to Sichuan Cancer Hospital from April 2016 to March 2023 were retrospectively analyzed.Their clinical data and survival were collected and summarized.Results Of the 45 patients,28.9%(13/45)were asymptomatic,97.2%(35/36)were positive for Epstein-Barr virus encoded small RNA,83.3%(10/12)were positive for programmed cell death-ligand 1(PD-L1),and the tumor proportion score(TPS)fluctuated between 10.0%to 95.0%.Genetic testing revealed 1 case of EGFR gene 19-Del mutation.Among the 45 patients,28 cases received surgical treatment,of which 16 received adjuvant chemotherapy,8 received adjuvant radiotherapy,and 2 received neoadjuvant chemotherapy.As of the follow-up cut-off date,33 patients survived and 12 patients died,with a median survival time not reached and an overall survival rate of 70.2%and 63.2%at 3 and 5 years,respectively.The 3-and 5-year disease-free survival rates were 67.2%and 57.6%for the 28 operated patients,respectively,and the progression-free survival rates were 58.3%and 29.2%for the 13 stageⅣpatients,respectively.Conclusions PPLELC is associated with Epstein-Barr virus infection and is a rare subtype of non-small cell lung cancer(NSCLC).Most patients do not have common gene mutations such as EGFR or ALK and have a high rate of positive PD-L1 expression.The disease is usually treated with a multi-method combination of mainly surgery,and is sensitive to radiotherapy and chemotherapy and has a better prognosis than other types of NSCLC.

关 键 词：肺淋巴上皮瘤样癌 肺癌 EB病毒 预后 

分 类 号：R734.2[医药卫生—肿瘤]