■肺肉瘤样癌(PSC)是高度恶性的非小细胞肺癌(NSCLC)的一个子集,侵略性,和异质特征。患有这种疾病的患者约占肺癌病例的0.1-0.4%。缺乏对PSC的基本生物学和临床治疗方法的全面总结,这意味着对这种罕见疾病的系统认识和理解有限。本文概述了PSC的生物学特性,并系统总结了该疾病患者可用的各种治疗策略。
■对于这篇叙述性评论,我们通过在PubMed数据库中搜索1990年7月16日至2023年8月29日发表的文章,检索了与PSC的基础生物学和临床治疗方法相关的文献.使用以下关键词:“肺肉瘤样癌”,“基因突变”,“免疫微环境”,“缺氧”,“血管生成”,“总生存率”,\"手术\",“放射治疗”,“化疗”,和“免疫检查点抑制剂”。
■经典PSC包括上皮和肉瘤样成分,大多数研究表明了一个共同的起源。PSC比其他类型的NSCLC表现出更高的肿瘤突变负荷(TMB)和突变频率。PSC的肿瘤微环境(TME)以缺氧为特征,代谢亢进,程序性细胞死亡蛋白1/程序性细胞死亡-配体1表达升高,和高免疫细胞浸润。晚期PSC的治疗策略主要基于传统的NSCLC治疗,但是PSC表现出对化疗和放疗的抵抗力。基因组测序的进步引入了靶向治疗作为突变阳性PSC病例的选择。此外,由于PSC免疫微环境的特点,许多患者对免疫疗法有积极的反应,展示了其管理PSC的潜力。
■尽管一些研究已经检查并评估了PSC的TME,这些在数量和质量上都是有限的,为PSC的临床治疗策略研究提出了挑战。随着新技术的出现和临床研究的进步,例如,savolitinib对MET外显子14跳跃突变阳性PSC患者的临床研究显示出有希望的结果,人们热切期待对PSC进行更深入的研究。
UNASSIGNED: Pulmonary sarcomatoid carcinoma (PSC) is a subset of non-small cell lung cancer (NSCLC) with highly malignant, aggressive, and heterogeneous features. Patients with this disease account for approximately 0.1-0.4% of lung cancer cases. The absence of comprehensive summaries on the basic
biology and clinical treatments for PSC means there is limited systematic awareness and understanding of this rare disease. This paper provides an overview of the biological characteristics of PSC and systematically summarizes various treatment strategies available for patients with this disease.
UNASSIGNED: For this narrative review, we have searched literature related to the basic
biology and clinical treatment approaches of PSC by searching the PubMed database for articles published from July 16, 1990 to August 29, 2023. The following keywords were used: \"pulmonary sarcomatoid carcinoma\", \"genetic mutations\", \"immune microenvironment\", \"hypoxia\", \"angiogenesis\", \"overall survival\", \"surgery\", \"radiotherapy\", \"chemotherapy\", and \"immune checkpoint inhibitors\".
UNASSIGNED: Classical PSC comprises epithelial and sarcomatoid components, with most studies suggesting a common origin. PSC exhibits a higher tumor mutational burden (TMB) and mutation frequency than other types of NSCLC. The tumor microenvironment (TME) of PSC is characterized by hypoxia, hypermetabolism, elevated programmed cell death protein 1/programmed cell death-ligand 1 expression, and high immune cell infiltration. Treatment strategies for advanced PSC are mainly based on traditional NSCLC treatments, but PSC exhibits resistance to chemotherapy and radiotherapy. The advancement of genome sequencing has introduced targeted therapies as an option for mutation-positive PSC cases. Moreover, due to the characteristics of the immune microenvironment of PSC, many patients positively respond to immunotherapy, demonstrating its potential for the management of PSC.
UNASSIGNED: Although several studies have examined and assessed the TME of PSC, these are limited in quantity and quality, presenting challenges for research into the clinical treatment strategies for PSC. With the emergence of new technologies and the advancement of clinical research, for example, savolitinib\'s clinical study for MET exon 14 skipping mutations positive PSC patients have shown promising outcomes, more in-depth studies on PSC are eagerly anticipated.