Abstract
e20541 Background: Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is an extremely rare subtype of non-small cell lung cancer (NSCLC) and there are no established treatment protocols due to its rarity. At present there are only limited studies about the molecular and clinical characteristics of PPLELC and the results are often inconsistent. Methods: The study collected patients with PPLELC who were admitted to Zhejiang Cancer Hospital from 2009 to 2020 retrospectively. Next-generation sequencing (NGS) was performed to obtain a more comprehensive mutation profile of PPLELE, tumor mutation burden (TMB) level and the expression of programmed cell death 1 ligand 1(PD-L1) was detected for 7 patients. In addition, we review the studies concerning the immunotherapy of advanced PPLELC. Results: 18 patients diagnosed as PPLELC pathologically were collected. 71.4% (5/7) patients expressed PD-L1 (≥ 1%) and the median TMB was 2.5 mutations/Mb. TP53 (43%) and CYLD (43%) were the two most common mutations and other variants included LRIG1 (14%), PTPRT (14%), PPP2R2A (14%). 15 patients survived, 3 patients died and 1 patient was lost after a followed up time for 8.8 to 138 months. A total of 11 cases of advanced PPLELC patients who received immunotherapy were analyzed retrospectively 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 time of advanced PPLELC.Whether the immunotherapy efficacy of PPLELC can be predict by PD-L1 and TMB is worthy of further clinical research. CYLD genetic alterations may participate in Epstein-Barr virus (EBV) -mediated tumorigenesis in PPLELC and expected to provide a new therapeutic target.
Highlights
Lymphoepithelioma-like carcinoma (LELC) is a rare malignant tumor, which shares similar histology with undifferentiated nasopharyngeal carcinoma (NPC)
Palliative therapy, including concurrent chemoradiotherapy, was undertaken in four patients while one patient did not receive any treatment after diagnosis
The information of 11 immunotherapy patients (10 Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) patients involved in previous studies (Kim et al, 2016; Kumar et al, 2017; Narayanan et al, 2019; Qiu et al, 2019; Zhou et al, 2019; Tang et al, 2020; Xie et al, 2020) and one patient received nivolumab in the current study marked as “IP”) were collected retrospectively (Table 2). 4/11 (36.4%) patients achieved partial response (PR), 6/11 (54.5%) patients achieved stable disease (SD), and 1/11 (9.1%) patients achieved progressive disease (PD)
Summary
Lymphoepithelioma-like carcinoma (LELC) is a rare malignant tumor, which shares similar histology with undifferentiated nasopharyngeal carcinoma (NPC). It occurs in the submandibular gland, parotid gland, thymus, lung, stomach, uterus, bladder, and skin (Bégin et al, 1987). Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare lung tumor with specific clinicopathological characteristics (Liang et al, 2012), affecting younger patients (51–55-year-old), mostly non-smokers, and mainly Asian females and women from Southern China (Huang et al, 2007; Sun et al, 2014; Jiang et al, 2016). The efficacy of nivolumab in the treatment of advanced NSCLC is significantly related to tumor mutation burden (TMB), and it is more effective in patients with high TMB compared to chemotherapy (Carbone et al, 2017; Peters et al, 2017). The TMB of PPLELC is low, a large amount of gene copy number variations (CNVs), especially 11q13.3 amplification and 9p21.3 deletion, have been observed (Hong et al, 2019)
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