Abstract

Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare subtype of non-small cell lung cancer (NSCLC) for which there is currently no recognized treatment. Recently, favorable immune checkpoint blockade responses have been observed in PPLELC. This study aimed to review the effects of this regimen in patients with advanced PPLELC. PPLELC patients treated with immune checkpoint inhibitors at West China Hospital between January 2008 and December 2019 were retrospectively identified. Demographic parameters and antitumor treatment details were retrieved and reviewed. Among 128 patients diagnosed with PPLELC, 5 who received immune checkpoint inhibitors at advanced stages were included in the analysis. All of these patients were female nonsmokers with a median age of 55.6 (range 53-58) years at diagnosis. Their median PD-L1 expression was 40% (range, 30-80%). Although the patients underwent surgeries, chemotherapy and radiotherapy, all the treatments failed. Immune checkpoint inhibitors were administered palliatively, and three patients responded favorably, with the best overall response being partial remission (PR). Thus, immune checkpoint inhibitors may be a promising treatment for advanced PPLELC, and large clinical trials are warranted to obtain more evidence regarding this regimen.

Highlights

  • Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare subtype of non-small cell lung cancer (NSCLC) that was first described by Begin et al in 1987 [1]

  • We reviewed the efficacy of immune checkpoint inhibitors in patients with PPLELC, and based on our preliminary experience, most patients responded favorably to the PD1/progressive disease (PD)-L1 blockade therapy

  • The features of PPLELC that distinguish it from other subtypes of nonsmall cell lung cancer (NSCLC) indicate that PPLELC is a unique subtype of lung cancer

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Summary

Introduction

Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare subtype of non-small cell lung cancer (NSCLC) that was first described by Begin et al in 1987 [1]. Programmed cell death-1 (PD-1) is a member of the B7 family that is expressed by activated T cells along with its ligand, i.e., programmed death-ligand 1 (PD-L1), to mediate immunoregulation [4]. PD-L1 is another immune checkpoint cell-surface protein that is expressed by tumor cells and host cells [5]. The interaction between PD-1 in T cells and PD-L1 in tumor cells leads to inhibition of the proliferation of activated T cells [6]. The inhibition of this interaction in vivo contributes to the enhancement of T-cell responses and can have antitumor activity [7]

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