Gong Chen, Xiong Huihua, Qin Kai, Wang Jianhua, Cheng Yi, Zhao Jing, Zhang Jing
Department of Oncology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Oncol. 2022 Sep 23;12:1017026. doi: 10.3389/fonc.2022.1017026. eCollection 2022.
Pulmonary sarcomatoid carcinoma (PSC) is a rare subset of NSCLC that accounts for about 0.5-1% of all primary lung carcinoma, and its malignant biological behavior is more aggressive than other pathological types of lung cancer. Recent studies have reported a variety of gene mutations associated with the occurrence, development and treatment of PSC, especially the mesenchymal-epithelial transition () proto-oncogene alterations, including the exon 14 (ex14) skipping mutations as well as the amplification and overexpression of gene, which are associated with molecularly targeted therapy for PSC. ex14 skipping mutation is the most common and well-studied mutation type, occurring in about 22-31.8% of PSC patients, while the prevalence of amplification is reported as 4.8-13.6% and ovexpression is about 20.2%. Molecular pathology tests, including IHC and NGS, are valuable in determining the prognosis of patients with PSC and helping to determine the treatment. The existing clinical data have confirmed the efficacy of -TKI in PSC patients with alteration, among which the clinical study of Savolitinib has enrolled the largest proportion of PSC patients and achieved relatively good efficacy, but more clinical researches are still needed. The multi-disciplinary team may maximize the optimal treatment options for patients with the advanced PSC.
肺肉瘤样癌(PSC)是NSCLC的一个罕见亚型,约占所有原发性肺癌的0.5%-1%,其恶性生物学行为比其他病理类型的肺癌更具侵袭性。最近的研究报道了多种与PSC的发生、发展和治疗相关的基因突变,尤其是间充质-上皮转化()原癌基因改变,包括外显子14(ex14)跳跃突变以及基因的扩增和过表达,这些与PSC的分子靶向治疗相关。ex14跳跃突变是最常见且研究最多的突变类型,约22%-31.8%的PSC患者发生该突变,而基因扩增的发生率据报道为4.8%-13.6%,过表达约为20.2%。分子病理学检测,包括免疫组化(IHC)和二代测序(NGS),对于确定PSC患者的预后和帮助确定治疗方案很有价值。现有临床数据证实了-TKI在有改变的PSC患者中的疗效,其中赛沃替尼的临床研究纳入的PSC患者比例最大且取得了相对较好的疗效,但仍需要更多的临床研究。多学科团队可为晚期PSC患者最大限度地提供最佳治疗方案。