Primary pulmonary lymphoepithelioma-like carcinoma

Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is an extremely rare subtype of non-small-cell lung cancer (NSCLC) but differs from other NSCLC types. Most patients with PPLELC have almost no obvious clinical manifestations, and are often diagnosed in the advanced stage. Complete resection is the standard treatment for cases with early stage disease, while chemotherapy and radiotherapy are effective to some extent in late stage disease. High expression of PD-L1 in PPLELC tumor cells confers immunotherapy an important role in the treatment of PPLELC, and programmed death-1 (PD-1) inhibitors such as nivolumab and pembrolizumab may be optimal options for PPLELC patients. This review focuses on the clinical and pathologic features, treatment, and prognosis of PPLELC.