Abstract
Pulmonary lymphoepithelioma carcinoma (PLEC), a rare non-small cell lung cancer subtype strongly associated with Epstein-Barr virus (EBV) infection, currently lacks a standardized treatment regimen. Conventional platinum-based systemic chemoradiotherapy remains the primary approach. In recent years, its distinctive pathological features and tumor immune microenvironment have established a compelling rationale for immune checkpoint inhibitors (ICIs). Clinical studies have demonstrated promising efficacy and acceptable safety of ICIs, both as monotherapy and in combination with chemotherapy, in patients with advanced and resectable PLEC. Neoadjuvant immunotherapy has also emerged as a viable strategy to downstage tumors and improve resectability in operable cases. However, the rarity of PLEC has limited the availability of large-scale prospective trials, and current evidence is predominantly derived from retrospective analyses. Key challenges, including predictive biomarkers, mechanisms of immunotherapy, and optimal combination strategies, remain to be addressed. This review systematically summarizes the current clinical evidence, molecular mechanisms, and immune microenvironment characteristics of PLEC, and discusses future research directions.
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