摘要目的:总结肺肉瘤样癌（PSC）患者的临床特征、治疗及转归，以提高临床对PSC的认识。方法:回顾性分析2015年1月1日至2023年11月30日，在北京协和医院住院诊治、经病理确诊为PSC患者43例的临床资料，其中男32例，女11例，年龄26~87（62.79±9.59）岁。根据是否行根治性切除手术分为可手术切除组和不可手术组，比较2组患者肿瘤的特点及治疗方式。通过Kaplan-Meier法绘制生存曲线比较2组患者的预后。结果:纳入的43例PSC患者中31例吸烟。肿瘤以周围型更常见，影像多以实性软组织团块影为主要表现，14例肿块最大径>5 cm。在23例完善NGS基因检测的患者中，KRAS突变率为43.5%，TP53突变率30.4%，MET突变率为8.7%，均为MET-14外显子跳跃突变。13例进行了PD-L1表达检测，其中10例高表达。43例平均OS为24.6个月（13.0~52.7个月）。其中，22例行根治性肺叶切除加纵隔淋巴结清扫术，13例随访期间出现术后复发，7例死亡；平均DFS为12.3个月，未达到平均OS，1年OS率为77.3%。21例处于不可手术切除的局部晚期或晚期，15例死亡；平均PFS为2.5个月，平均OS为6.2个月，1年OS率为42.9%。17例接受了免疫治疗，1例接受了MET抑制剂谷美替尼靶向治疗。结论:PSC在老年、吸烟、男性中发生率更高，是一种恶性程度高、预后较差的NSCLC，基于其分子生物学的特征，可进行PD-L1和肿瘤分子检测来指导治疗。

abstractsObjective:To summarize the clinical characteristics, treatment, and outcomes of patients with pulmonary sarcomatoid carcinoma (PSC) in order to improve clinicians′ understanding of this disease.Methods:The clinical data of patients diagnosed with PSC in our hospital from January 1, 2015 to November 30, 2023 were retrospectively analyzed. According to whether radical resection was performed, the patients were divided into resectable group and unresectable group. The characteristics and treatments of PSC in different groups were compared. The survival curves were drawn by Kaplan-Meier method to compare the prognosis of different groups of patients.Results:A total of 43 PSC patients were included, including 32 males, with an average age of (62.79±9.59) years, and 31 smokers. Peripheral-type tumors were more common, with imaging showing predominantly solid soft tissue masses, and the maximum diameter of the tumor was more than 5 cm in 14 patients. Among the 23 patients who underwent NGS gene testing, the KRAS mutation rate was 43.5%, the TP53 mutation rate was 30.4%, and the MET mutation rate was 8.7%, all of which were MET-14 exon skipping mutations. PD-L1 expression was detected in 13 patients, 10 of whom showed high expression. The median overall survival (mOS) of the 43 patients with PSC was 24.6 months (13.0-52.7 months). Among them, 22 patients underwent radical lobectomy plus mediastinal lymph node dissection, 13 patients had postoperative recurrence, and 7 patients died during follow-up. The median disease-free survival (mDFS) was 12.3 months, the mOS was not achieved and the 1-year OS rate was 77.3 %. Twenty-one patients had unresectable locally advanced or advanced stage, and 15 patients died. The mDFS was 2.5 months, the mOS was 6.2 months, and the 1-year OS rate was 42.9 %. Seventeen patients received immunotherapy, and 1 patient received targeted therapy with the MET inhibitor glumetinib.Conclusions:PSC has a higher incidence in the elderly, smokers, and males, is highly malignant and has a poor prognosis. Based on its molecular biological characteristics, PD-L1 expression and tumor molecular detection can be performed to guide treatment options.