Primary lymphoepithelioma-like carcinoma of the lung: An unusual cancer and clinical outcomes of 14 patients

Abstract

Abstract. Advanced lung cancer is considered to exhibit a poor prognosis; however, the pulmonary lymphoepithelioma-like carcinoma (LELC), a rare subtype of non-small cell lung cancer (NSCLC), exhibits an improved prognosis, compared with non-LELC. The present study aimed at investigating the clinical manifestation, imaging characteristics, pathology, tumor markers, treatment and prognosis of primary LELC of the lung. A total of 14 patients with pulmonary LELC were confirmed by surgery and pathology. Clinical data of those patients were retrospectively reviewed including age, sex, smoking history, symptoms, computed tomography (CT) results, Epstein-Barr virus-encoded RNA (EBER) status, treatment and outcomes. In the present study, there were 7 males and 7 females who ranged in age between 22 and 64 years (mean, 51.21±11.37 years) and who all were from eastern China. The tumor-node-metastasis stage ranged between stages I and IV, with 71.43% of the patients at advanced stage (stages III and IV). The results of the present study identified 100% positive expression of EBER. Tumors located centrally were of significantly increased size, compared with peripheral tumors (P<0.05), and lymphadenopathy was more common in patients with advanced stage (P<0.05). The majority of patients were treated with surgery, platinum-based chemotherapy or radiotherapy. At time of writing, 12 patients were alive and the longest survival time was 60 months. Pulmonary LELC typically affected young patients and was not associated with smoking history; however, pulmonary LELC was associated with Epstein-Barr virus infection in the Asian population. The majority of patients were in early or locally advanced stages and exhibit an improved prognosis compared with other types of NSCLC. Pulmonary LELC was sensitive to chemotherapy and surgery, with postoperative chemotherapy-based multimodality treatment recommended.

Pulmonary Lymphoepithelioma-like Carcinoma

“…15 There is no sex predilection in most of the studied populations. 2,11,12,[14][15][16] The age at diagnosis usually ranges from 35 to 85 years, 1,2,10,12,13,17 with the mean/median age between 51 and 57 years. 2,10,12,13,17 Overall, patients with pulmonary LELC have been found to be younger than those with other NSCLCs.…”

Low frequency of mutation of epidermal growth factor receptor (EGFR) and arrangement of anaplastic lymphoma kinase (ALK) in primary pulmonary lymphoepithelioma‐like carcinoma

“…ALK alteration was detected positive in three PLELC patients in our study, while only one was reported in the literature by Ose et al 38 The variant rates may be due to the small sample size of each study which warrants further investigation. In the study by Gou and Wu which demonstrated the frequencies of driver mutations in nonsmall cell lung cancer in China, 19 the mutation rates of EGFR were 48.4% (675/1795), 4.3% (9/208), and 28.2% (570/2021) in lung adenocarcinoma, squamous cell cancer 36 Hong Kong, China 11 NA 0 NA NA Liang et al 8 Guangzhou, China 52 0/11 NA NA NA Liu et al 29 Guangzhou, China 32 0 NA NA NA Wang et al 28 Guangzhou, China 42 1 0 NA NA Fang et al 37 Guangzhou, China 113 2 0 NA NA Chang et al 30 Tai Wan, China 66 8 0 0 0 (BRAF) Ose et al 38 Osaka, Japan 1 0 1 NA NA Hong et al 31 Guangzhou, China 91 0 0 NA 14 (P53) Lin et al 39 Guangzhou, China 39 0/19 NA NA NA Lin et al 40 Fuzhou, China 20 0/3 0/2 0/1 NA Xie et al 10 Guangzhou, China 429 2 0 0 NA Zhou et al 41 Guangzhou, China 27 0 0 1 7 (P53) Yin et al 42 Guangzhou 43 An et al reported that the mutation rate of driver genes might differ among different histology subtypes and smoking status. 44 EGFR and ALK alteration is rare in PLELC.…”

Plasma Epstein-Barr Virus-Deoxyribonucleic Acid Copy Number Predicts Disease Progression in Stage I–III Pulmonary Lymphoepithelioma-Like Carcinoma

“…Pulmonary lymphoepithelioma-like carcinoma (LELC) is a rare subtype of non-small cell lung cancer (NSCLC), accounting for < 1% of all histologic type of lung cancers. So far only ∼600 cases have been reported in the English-language literature (1)(2)(3)(4)(5)(6)(7). Histologically, the disease is characterized by epithelial tumor cells nests surrounded or infiltrated by numerous lymphocytes (1) ( Figure 1A).…”

“…Based on the experience on nasopharyngeal cancer, the potential diagnostic, prognostic and predictive role of plasma EBV-DNA copy number in pulmonary LELC triggered much interest. Because of the rarity of pulmonary LELC, previous studies usually focused on small, heterogeneous cohorts with different tumor stage and inconsistent treatments (2)(3)(4). There lacks a universally accepted threshold of EBV-DNA copy number suggesting immediate disease progression for the patients after radical treatment.…”