Objective  To investigate the clinical characteristics, treatment, and prognosis of primary pulmonary lymphoepithelioma-like carcinoma.

Methods  Clinical data of 39 patients with primary pulmonary lymphoepithelioma-like carcinoma and were hospitalized in Xiamen Branch, Zhongshan Hospital, Fudan University and Zhongshan Hospital, Fudan Universtity from January 2015 to December 2019 were retrospectively analyzed.

Results  Symptoms were not specific, and 48.7%(19/39) patients were found by physical examination. 83.9% (26/31) patients were PD-L1 positive, and 97.2% (35/36) patients were Epstein-Barr virus encoded small RNA (EBER) positive. Genetic testing found that 1 patient had ROS-1 rearrangement, but no EGFR mutation and ALK rearrangement were detected. 64.1% (25/39) patients underwent surgical treatment, and the patients who could not be operated were treated with multidisciplinary comprehensive treatment strategy. The median follow-up time was 33.7(23.4, 47.7) months, and the median survival time was not reached in all patients. Four patients died, of whom, one patient had a ROS-1 mutation with a survival time of 24.4 months; the survival time of the other 3 patients was 49.7, 25.3, 69.7 months respectively. Correlation analysis showed that the expression of PD-L1 was not an independent risk factor of survival time.

Conclusion  Primary pulmonary lymphoepithelioma-like carcinoma is a rare type of primary lung cancer. It's occurrence is closely related to the Epstein-Barr virus. Lung cancer driver gene mutations are rare. A multidisciplinary comprehensive diagnosis and treatment strategy based on surgery is offen adopted. Most patients have a good prognosis.