探讨国内外30余年来原发性肺淋巴上皮瘤样癌的临床特点。

通过中国知网、万方数据库、维普中文科技期刊、PubMed等数据库，以“肺淋巴上皮瘤样癌；primary pulmonary lymphoepithelioma-like carcinoma”为关键词，对1988年1月~2020年6月30余年间发表的中英文献进行检索，提取相关资料并进行分析。

最终纳入文献44篇，共179例患者，其中男83例，女96例，无吸烟史132例。最常见的临床表现为咳嗽、咳痰，部分患者无症状，由体检发现；179例LELC患者中，173例为单发病灶，左肺93例，右肺82例，其中左下肺最多占31.8%，右中肺占24.6%；EB病毒血清学检查阳性率为82.4%，病理学可见多巢状肿瘤细胞，核大呈空泡状，肿瘤间质有丰富的淋巴细胞浸润，免疫组化CK阳性率为100%（75/75），鳞癌标记物CK5/6、P63、P40阳性率分别为94.4%（68/72）、88.6%（62/70）、83.9%（26/31）；EBER原位杂交阳性率95.5%（127/133）；治疗方法包括手术、放化疗、免疫治疗等，患者最长生存时间134个月。

PPLELC好于中年、不吸烟人群，症状以咳嗽、咳痰最常见，部分无症状，病灶多单发，好发于左下肺和右中肺，与一般肺癌不易鉴别；确诊依靠病理学，免疫组化易与鳞癌混淆，EB病毒的血清学检测阳性率高，病灶EBER原位杂交有助于确诊；主要治疗手段为手术、放化疗等，靶向治疗获益少，免疫治疗有待研究。

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.