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目的:探讨支气管肺大细胞神经内分泌癌（LCNEC）的临床表现、影像和分子病理特征。方法:回顾性分析2011—2021年郑州大学第一附属医院216例LCNEC患者的临床资料，分析其临床表现、肿瘤位置及大小、CT影像特征、免疫组化及分子病理特点，并与同期确诊的115例复合型小细胞癌（M-SCLC）对比。结果:LCNEC患者中，男190例，女26例，年龄33~85岁，中位年龄65岁。首诊症状以咳嗽［106例（49.1%）］与痰中带血［48例（22.2%）］为主。肿瘤长径中位数4.7 cm，其中结节型55例（25.5%），肿块型161例（74.5%）。CT结果显示，LCNEC病变呈软组织密度，轻度强化的比例低于M-SCLC组（52.3% 比74.8%，n P<0.001）。相反，LCNEC患者瘤体中出现坏死（87.0% 比 58.3%，n P<0.001）及钙化（26.9% 比 2.6%，n P<0.001）的比例高于M-SCLC组。免疫组化结果显示，LCNEC中的CK阳性率高于M-SCLC（99.0% 比 90.5%，n P<0.05），而TTF-1阳性率低于M-SCLC（51.6% 比 67.0%，n P25个突变/MB的比例为27.3%。n 结论:LCNEC缺乏特异性临床表现，CT影像和M-SCLC有特征性鉴别点，分子病理有一定的突变频谱，确诊仍需要病理联合免疫组化综合判读，与M-SCLC的鉴别主要依靠光镜下细胞大小及核染色质模式。“,”Objective:To investigate the clinical manifestations, imaging, pathological and molecular features of bronchopulmonary large-cell neuroendocrine carcinoma (LCNEC).Methods:The clinical data of 216 LCNEC patients in the First Affiliated Hospital of Zhengzhou University from 2011 to 2021 were analyzed retrospectively. The clinical manifestations, tumor location and size, characteristics of CT images, immunohistochemical and molecular pathological features were analyzed and compared with 115 cases of mixed small cell carcinoma (M-SCLC) diagnosed in the same period.Results:Among the 216 LCNEC patients, there were 190 males and 26 females, with a median age of 65 years. The first symptoms of the patients were mainly cough (106 cases, 49.1%) and bloody sputum (48 cases, 22.2%). The median tumor length were 4.7cm, including 55 cases of nodular type (25.5%) and 161 cases of mass-forming type (74.5%). CT imaging results showed that LCNEC lesions had soft tissue density, and the proportion of slight enhancement lesions was significantly lower than that in M-SCLC group (52.3% vs 74.8%, n P<0.001). In contrast, the proportion of necrosis (87.0% vs 58.3%, n P<0.001) and calcification (26.9% vs 2.6%, n P<0.001) in LCNEC patients was significantly higher than that in M-SCLC group. Immunohistochemical results showed that the positive rate of CK in LCNEC was significantly higher than that in M-SCLC n (99.0 % vs 90.5%, n P<0.05n ), while the positive rate of TTF-1 was significantly lower than that in M-SCLC (51.6% vs 67.0%, n P<0.05n ). In LCNEC group, the proportion of patients with Ki-67 positive index between 50% and 80% was significantly higher than that of M-SCLC (41.2% vs 25.2%), while the proportion between 80% and 100% was lower than that of M-SCLC (51.9% vs 72.2%). There was no significant difference in the positive rates of CD56 (91.7% vs 94.6%, n P=0.336), Syn (83.8% vs 84.7%, n P=0.838) and CgA (54.8% vs 50.0%, n P=0.632) in both tumor types. Molecular pathology results showed that frequent mutatios were TP53 (54.5%), RB1 (36.4%), KEAP1 (18.2%), MYC(18.2%), and PTEN (14.3%), and the rate of tumor mutation burden which is more than 25 mutation/Mb was 27.3%.n Conclusions:LCNEC lacks specific clinical manifestations. CT imaging is powerful in distinguishing LCNEC from M-SCLC. LCNEC contains a specific mutation spectrum. Pathology combined with immunohistochemical staining is still the gold standard for LCNEC diagnosis, and the differentiation from M-SCLC mainly depends on cell size and nuclear chromatin pattern with light microscopy.