摘要
背景与目的淋巴上皮瘤样癌为罕见的上皮肿瘤,多来源于鼻咽部,也发生在前肠起源器官,如肺、胃、唾液腺和胸腺。原发性肺淋巴上皮瘤样癌(primary pulmonary lymphoepithelioma-like carcinoma, PPLELC)是一种罕见的非小细胞肺癌(non-small cell lung cancer, NSCLC),约占肺癌的0.9%。本文目的在于探讨PPLELC的临床病理特点、治疗方法以及预后情况。方法回顾性分析湖南省肿瘤医院2013年10月-2016年6月收治的PPLELC患者8例,对其临床资料及预后进行临床分析,以深入研究这种罕见的肺癌亚型。结果 8例患者中,62.5%(5/8)为女性,中位年龄为51.5岁(41岁-64岁),大多数(87.5%)患者不吸烟,50.0%患者无症状,约37.5%为I期,50.0%为Ⅲ期,12.5%为Ⅳ期。PPLELC的典型病理特征与未分化的鼻咽癌相同,其特点是分化较差的肿瘤细胞,具有大的泡状核和核仁突出,呈合胞体生长,伴有淋巴细胞浸润。免疫表型:癌细胞P63(100.0%, 6/6)、CK5/6(100.0%, 5/5)、CK(100.0%, 5/5)阳性。对8例患者的程序性死亡配体-1(programmed cell death-ligand 1, PD-L1)表达进行了检测,当≥5%的细胞膜染色时定义为PD-L1阳性,PD-L1表达频率为50.0%(4/8),肿瘤细胞阳性比例分数(tumor proportion score, TPS)波动在20.0%-70.0%。3例行表皮生长因子受体(epidermal growth factor receptor, EGFR)基因突变检测,结果呈阴性;2例行EML4-ALK融合基因检测,结果呈阴性;1例行KRAS、B-raf、C-kit、HER2、VEGFR1、VEGFR2检测,结果示KRAS野生型,B-raf、C-kit无突变,HER2、VEGFR1、VEGFR2中等表达。所有患者均接受了手术治疗,其中接受了辅助化疗者5例,其中Ib期2例,Ⅲa期2例,Ⅳ期1例;辅助放化疗者1例,分期为Ⅲa期,接受了新辅助化疗者1例,分期为Ⅲa期。截止至随访截止期,所有患者均存活,中位存活时间为57个月,3年和5年总生存率(overall survival, OS)分别为87.5%和50.0%;无病生存率(diseasefreesurvival,DFS)分别为87.5%和50.0%。结论 PPLELC是一种�
Background and objective Lymphoepithelioma-like carcinoma,an uncommon epithelial tumor,is mostly originated form the nasopharynx and also occurs in foregut-derived organs,such as lung,stomach,salivary gland,and thymus.Primary pulmonary lymphoepithelioma-like carcinoma(PPLELC)is a rare subtype accounting for around 0.9%of non-small cell lung cancer(NSCLC).We aimed to evaluate clinicopathological features,treatment modalities,and prognosis of PPLELC.Methods In the current study,a retrospective analysis on 8 patients diagnosed with PPLELC at Hunan Cancer Hospital between October 2013 and June 2016 was conducted with respect to their clinical characteristics and outcomes,in order to deeply investigate this rare subtype of lung cancer.Results In all 8 patients,62.5%(5/8)were female,and the median age was 51.5 years(range 41 years-64 years).The majority(87.5%)were never smokers and 50.0%were asymptomatic at diagnosis.About 37.5%presented with stage I disease,50.0%had stageⅢdisease and 12.5%had stageⅣdisease.Histologically,the tumor morphology was indistinguishable from undifferentiated carcinoma ofthe nasopharynx and were characterized by poorly differentiated tumor cells with large vesicular nuclei and prominent nucleoli showing syncytial growth patterns and accompanied by heavy lymphocytic infiltration.The tumor cells were presented as positive for P63(100.0%,6/6),CK5/6(100.0%,5/5),CK(100.0%,5/5).The expression of programmed cell death ligand 1(PD-L1)in 8 patients was detected.When membranous staining was present in≥5%of the cells,it was defined as PD-L1 positive.The PD-L1 expression frequency was 50.0%(4/8),and the tumor proportion score(TPS)fluctuated between 20.0%and 70.0%.Epidermal growth factor receptor(EGFR)mutations were detected in 3 cases and all tested samples were wild type;moreover,ALK rearrangement was negative in 2 patients with available data.KRAS,B-raf,C-kit,HER2,VEGFR1 and VEGFR2 were detected in 1 case.The results showed that KRAS was wild type,B-raf and C-kit mutation was negative,and HER2,VE
作者
赵惠
陈建华
Hui ZHAO;Jianhua CHEN(Department of Thoracic Medicine,Affiliated Cancer Hospital of Xiangya School of Medicine,Central South University,Hunan Cancer Hospital,Changsha 410006,China)
出处
《中国肺癌杂志》
CAS
CSCD
北大核心
2020年第3期168-175,共8页
Chinese Journal of Lung Cancer
关键词
淋巴上皮瘤样癌
肺肿瘤
临床病理
预后
Lymphoepithelioma-like carcinoma
Lung neoplasms
Clinicopathological features
Prognosis
