To summarize the clinical features of primary pulmonary lymphoepithelioma-like carcinoma by performing a literature review of case reported in the last 30 years.

Using "primary pulmonary lymphoepithelioma-like carcinoma" as the key word, CNKI (China National Knowledge Infrastructure), Wanfang Database, Viper database, and PubMed were searched for cases reported from January 1988 to June 2020.

A total of 44 articles involving 179 patients were included in this study. There were 83 male and 96 female patients women, and 132 patients had no smoking history. Cough and expectoration were the most common clinical manifestations. Some patients were asymptomatic and were discovered in the physical examination. Among the 179 LELC patients, 173 had a single lesion. There were 93 cases of left lung involvement and 82 cases of right lung involvement. Tumors occurred more frequently in the right middle lobe (24.6%) and left lower lobe (31.8%). The serological positive rate of EB virus was 82.4%, and the genetic positive rate was low. Pathology showed nests of tumor cells with large vacuolated nuclei with abundant lymphocytic infiltrates. The positive rate for CK was 100% (75/75). The positive rates for CK5/6, P63, and P40 were 94.4% (68/72), 88.6% (62/70), and 83.9% (26/31), respectively. The positive rate of EBER in situ hybridization (ISH) was 95.5% (127/133). Surgery and chemoradiotherapy were recommended. The longest survival was 134 months.

PPLELC occurs mostly in middle-aged people and non-smokers. The most common manifestations are cough and sputum, with some patients being asymptomatic. Tumors occur more frequently in the right middle lobe and left lower lobe, which are not easy to differentiate from common lung cancer types. The diagnosis of PPLELC depends on pathology, and immunohistochemistry may result in confusion with squamous cell carcinoma easily. The serological detection rate of EBV is high. Positive EBER ISH is helpful to diagnosis. The main therapeutic methods are surgery and chemoradiotherapy. Targeted therapy has little benefit and the efficacy of immunotherapy remains to be studied.