HFCAS OpenIR
Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma
Fan, Ying1,2,3,4; Shan, Qianyun1,2,3,5; Gong, Jiali1,2,3,5; Qin, Jing1,2,3; Lu, Hongyang1,2,3,4,5
2021-10-25
发表期刊FRONTIERS IN MOLECULAR BIOSCIENCES
通讯作者Lu, Hongyang(luhy@zjcc.org.cn)
摘要Objectives: Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is an extremely rare subtype of non-small cell lung cancer (NSCLC). Currently, there are no established treatment protocols due to rarity of the cancer. Thus, this study aimed to explore the molecular and clinical characteristics of PPLELC.

Study design and setting: Data from patients with PPLELC who were admitted to Zhejiang Cancer Hospital from August 2009 to September 2020 were retrospectively collected. Next-generation sequencing was performed to obtain a genomic profile and tumor mutation burden (TMB) value of patients with adequate tissue and divided them into two groups according to the expression level of PD-L1. The correlation of PD-L1 expression and the clinicopathological characteristics was evaluated by Pearson Chi-square test. Kaplan-Meier curves was applied to present the probability of survival between PD-L1 expression level and overall survival (OS). Moreover, the literature on the immunotherapy of advanced PPLELC published in PubMed between 2016 and 2020 were reviewed and the efficacy of immunotherapy were analyzed.

Results: A total of 18 patients pathologically diagnosed as PPLELC were included. After a follow-up period of 8.8-138 months, 14 patients survived, three patients died and one patient lost, the median OS was 45.3 months Seven samples (tissue-available) tested by NGS and the median TMB was 2.5 mutations/Mb. 19 somatic mutated genes were recognized and TP53 (43%) and CYLD (43%) were the two most commonly mutated genes. Only seven patients who underwent NGS were tested for PD-L1. Three patients with high PD-L1 expression (PD-L1 >= 50%) and four patients with low PD-L1 expression (PD-L1 <50%) were included. No significant correlation was observed between PD-L1 expression and clinical characteristics (age, gender, smoking status, tumor stage, lymph node metastasis) (p > 0.05) and OS (p = 1). What's more, 10 PPLELC patients involved in previous studies and one patient received nivolumab in the current study were collected retrospectively. 4/11 (36.4%) patients achieved PR, 6/11 (54.5%) patients achieved SD, and 1/11 (9.1%) patients achieved PD and the disease control rate (DCR) was 90.9%.

Conclusions: The prognosis of PPLELC is better than that of other NSCLC, and immunotherapy may be a promising treatment to prolong the survival of advanced PPLELC patients. Whether the immunotherapy efficacy of PPLELC can be predicted by PD-L1 and TMB needs further clinical investigation. CYLD genetic alterations may participate in Epstein-Barr virus-mediated tumorigenesis in PPLELC, providing a novel therapeutic target.

关键词PPLELC clinical characteristics molecular characteristics immunotherapy PD-L1 TMB
DOI10.3389/fmolb.2021.736940
关键词[WOS]COMPUTED-TOMOGRAPHY CHARACTERISTICS ; MUTATION STATUS ; PD-1 BLOCKADE ; NIVOLUMAB ; CANCER ; IMMUNOTHERAPY ; EXPRESSION ; FEATURES ; OUTCOMES ; P53
收录类别SCI
语种英语
资助项目Zhejiang Province Medical Science and Technology Project[2020ZH001] ; [e20541]
项目资助者Zhejiang Province Medical Science and Technology Project
WOS研究方向Biochemistry & Molecular Biology
WOS类目Biochemistry & Molecular Biology
WOS记录号WOS:000717349500001
出版者FRONTIERS MEDIA SA
引用统计
被引频次:4[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.hfcas.ac.cn:8080/handle/334002/126543
专题中国科学院合肥物质科学研究院
通讯作者Lu, Hongyang
作者单位1.Univ Chinese Acad Sci, Zhejiang Canc Hosp, Zhejiang Key Lab Diag & Treatment Technol Thorac, Canc Hosp, Hangzhou, Peoples R China
2.Univ Chinese Acad Sci, Zhejiang Canc Hosp, Dept Thorac Med Oncol, Canc Hosp, Hangzhou, Peoples R China
3.Chinese Acad Sci, Inst Basic Med & Canc IMBC, Hangzhou, Peoples R China
4.Wenzhou Med Univ, Clin Med Coll 1, Wenzhou, Peoples R China
5.Zhejiang Chinese Med Univ, Clin Med Coll 2, Hangzhou, Peoples R China
推荐引用方式
GB/T 7714
Fan, Ying,Shan, Qianyun,Gong, Jiali,et al. Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma[J]. FRONTIERS IN MOLECULAR BIOSCIENCES,2021,8.
APA Fan, Ying,Shan, Qianyun,Gong, Jiali,Qin, Jing,&Lu, Hongyang.(2021).Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma.FRONTIERS IN MOLECULAR BIOSCIENCES,8.
MLA Fan, Ying,et al."Molecular and Clinical Characteristics of Primary Pulmonary Lymphoepithelioma-Like Carcinoma".FRONTIERS IN MOLECULAR BIOSCIENCES 8(2021).
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