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Abstract：
BACKGROUND: 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.
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.
【中文题目：39例肺肉瘤样癌临床病理特征及预后分析】 【中文摘要：背景与目的 肺肉瘤样癌（pulmonary sarcomatoid carcinoma, PSC）是非小细胞肺癌（non-small cell lung cancer, NSCLC）的罕见类型，具有低发病率、高度恶性、强侵袭性、预后差的特点，当前无标准治疗方案。本研究拟通过收集PSC患者临床病理特征、当前诊治情况并分析预后因素，总结诊治经验，旨在提高临床对PSC的认识。方法 回顾性收集2013年12月至2023年12月于北京胸科医院确诊、接受治疗且临床资料完整的39例PSC患者的人口学信息、临床病理特征、肿瘤原发灶-淋巴结-转移（tumor-node-metastasis, TNM）分期和诊疗方案资料，并完成临床预后随访。应用Kaplan-Meier法进行单因素生存分析。结果 39例PSC患者年龄范围45-76岁，其中男性35例，女性4例，首诊临床表现缺乏特异性；20例患者接受手术治疗，19例患者行姑息性放化疗或对症支持治疗。患者1、5年生存率分别为61.90%、35.20%。单因素分析结果提示恶性肿瘤家族史、肿瘤部位、TNM分期、淋巴结转移、远处转移、是否手术、手术类型、治疗方案、细胞程序性死亡配体1（programmed cell death ligand 1, PD-L1）蛋白表达≥1%、间质上皮细胞转化因子（mesenchymal-epithelial transition factor, MET）通路异常与患者总生存期（overall survival, OS）有关（P<0.05）；多因素分析结果显示，淋巴结转移是患者OS的独立影响因素（P=0.037）。结论 PSC临床发病率低，多见于有吸烟史的老年男性。PD-L1蛋白表达≥1%及MET通路异常可提示患者不良预后，淋巴结转移是患者OS的独立危险因素。以手术为主的综合治疗是早期及局部晚期患者的主要治疗模式，靶向治疗和免疫治疗的临床应用价值有待进一步探索。 】 【中文关键词：肺肉瘤样癌；临床特征；病理特征；预后】.
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.
【中文题目：39例肺肉瘤样癌临床病理特征及预后分析】 【中文摘要：背景与目的 肺肉瘤样癌（pulmonary sarcomatoid carcinoma, PSC）是非小细胞肺癌（non-small cell lung cancer, NSCLC）的罕见类型，具有低发病率、高度恶性、强侵袭性、预后差的特点，当前无标准治疗方案。本研究拟通过收集PSC患者临床病理特征、当前诊治情况并分析预后因素，总结诊治经验，旨在提高临床对PSC的认识。方法 回顾性收集2013年12月至2023年12月于北京胸科医院确诊、接受治疗且临床资料完整的39例PSC患者的人口学信息、临床病理特征、肿瘤原发灶-淋巴结-转移（tumor-node-metastasis, TNM）分期和诊疗方案资料，并完成临床预后随访。应用Kaplan-Meier法进行单因素生存分析。结果 39例PSC患者年龄范围45-76岁，其中男性35例，女性4例，首诊临床表现缺乏特异性；20例患者接受手术治疗，19例患者行姑息性放化疗或对症支持治疗。患者1、5年生存率分别为61.90%、35.20%。单因素分析结果提示恶性肿瘤家族史、肿瘤部位、TNM分期、淋巴结转移、远处转移、是否手术、手术类型、治疗方案、细胞程序性死亡配体1（programmed cell death ligand 1, PD-L1）蛋白表达≥1%、间质上皮细胞转化因子（mesenchymal-epithelial transition factor, MET）通路异常与患者总生存期（overall survival, OS）有关（P<0.05）；多因素分析结果显示，淋巴结转移是患者OS的独立影响因素（P=0.037）。结论 PSC临床发病率低，多见于有吸烟史的老年男性。PD-L1蛋白表达≥1%及MET通路异常可提示患者不良预后，淋巴结转移是患者OS的独立危险因素。以手术为主的综合治疗是早期及局部晚期患者的主要治疗模式，靶向治疗和免疫治疗的临床应用价值有待进一步探索。 】 【中文关键词：肺肉瘤样癌；临床特征；病理特征；预后】.
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