肺肉瘤样癌（PSC）是非小细胞肺癌（NSCLC）的一种罕见亚型，具有发病率低、恶性率高、侵袭性强、预后差等特点。迄今为止，还没有标准化的治疗方法。本研究旨在总结PSC的临床病理特点、诊断方法、治疗方案及预后因素，以更好地认识该疾病，积累更多的临床经验。共纳入北京胸科医院诊断并接受治疗的39例PSC患者。回顾性招募2013年12月至2023年12月期间的患者，仔细收集人口学特征、临床病理特征、肿瘤淋巴结转移（TNM）分期、诊断方法和治疗方案等信息。同时，也进行了跟踪调查。采用Kaplan-Meier法分析该病的预后因素。本研究中的PSC患者年龄为45~76岁，其中男性35例，女性4例。初次诊断时PSC无特异性临床表现。 39例患者中，20例接受手术切除，19例接受姑息性放化疗或对症支持治疗。 1年和5年生存率分别为61.90%和35.20%。单因素分析显示癌家族史、原发部位、TNM分期、淋巴结转移、远处转移、是否接受手术切除、手术方式、治疗方案、肿瘤组织程序性细胞死亡配体1（PD-L1）表达≥ 1%和间充质-上皮转化因子（MET）通路异常与患者的总生存期（OS）相关（P<0.05）。在随后的多变量分析中，淋巴结转移成为预测较差 OS 的唯一独立预后因素（P=0.037）。PSC 在临床实践中很少见，常见于有吸烟史的老年男性。肿瘤组织PD-L1表达≥1%且MET异常可预测PSC预后较差，淋巴结转移被确定为PSC的独立预后因素。手术切除和辅助药物治疗是早期和局部晚期患者的基石，分子靶向治疗和免疫治疗在PSC中的临床效用有待进一步研究。

Pulmonary sarcomatoid carcinoma (PSC) is a rare subtype of non-small cell lung cancer (NSCLC), which is featured by low incidence, high malignancy rate, robust aggressive behavior and inferior prognosis. To date, there is no standardized treatment. The aim of this study is to better understand and accumulate more clinical experience of the disease by summarizing the clinicopathological features, diagnosis methods, therapeutic regimen and prognostic factors of PSC.A total of 39 patients with PSC who diagnosed and received treatment in Beijing Chest Hospital from December 2013 to December 2023 were retrospectively recruited, and information including demographic characteristics, clinicopathological features, tumor-node-metastasis (TNM) stage, diagnosis method and therapeutic regimen were carefully collected. Meanwhile, follow-up was conducted. Kaplan-Meier method was used to analyze the prognostic factors of the disease.The PSC patients in this study ranged in age from 45 to 76 years old, including 35 males and 4 females. There were no specific clinical manifestations of PSC at initial diagnosis. Among the 39 patients, 20 underwent surgical resection and 19 received palliative chemoradiation or symptomatic supportive treatment. The 1-year and 5-year survival rates were 61.90% and 35.20% respectively. Univariate analysis indicated that family history of carcinoma, primary tumor site, TNM stage, lymph node metastasis, distant metastasis, whether or not received surgical resection, surgical method, treatment regimens, tumor tissue programmed cell death ligand 1 (PD-L1) expression ≥1% and mesenchymal-epithelial transition factor (MET) pathway abnormalities were correlated with the overall survival (OS) of patients (P<0.05). In the subsequent multivariate analysis, lymph node metastasis emerged as the only independent prognosticator in predicting inferior OS (P=0.037).PSC is rarely seen in clinical practice and commonly occurs in elder men with smoking history. Tumor tissue PD-L1 expression ≥1% and MET abnormalities may predict inferior prognosis of PSC and lymph node metastasis was determined as the independent prognosticator of PSC. Surgical resection along with adjuvant medical treatment is the cornerstone for early and locally advanced patients, and the clinical utility of molecular targeting therapy and immunotherapy in PSC needs to be further investigated.