肺原发性涎腺型肿瘤的诊疗进展  

Advances in diagnosis and treatment of primary salivary gland-type tumors of the lung

作　　者：孙先睿 高若淋 庄威 吴鑫宇 仲佳 Sun Xianrui;Gao Ruolin;Zhuang Wei;Wu Xinyu;Zhong Jia(Department of Medical Oncology,the First Affiliated Hospital of China Medical University,Shenyang 110179,China;Department of Medical Oncology,National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100021,China)

机构地区：[1]中国医科大学附属第一医院肿瘤内科,沈阳110179 [2]国家癌症中心国家肿瘤临床医学研究中心中国医学科学院北京协和医学院肿瘤医院内科,北京100021

出　　处：《中国肿瘤临床与康复》2025年第3期147-159,共13页Chinese Journal of Clinical Oncology and Rehabilitation

基　　金：国家自然科学基金(82303969)。

摘　　要：肺原发性涎腺型肿瘤(PSGT)起源于大气道的黏膜下腺体,是一种罕见的肺部肿瘤,发病率在所有肺原发性肿瘤中不足0.2%。PSGT的诊断需与肺部其他原发肿瘤及肺外来源转移性疾病相鉴别,尤其是头部和颈部的涎腺。分子改变在PSGT的诊断中具有重要作用,如黏液表皮样癌中的CREB调节转录辅激活因子1(CRTC1)和智者基因样2(MAML2)重排、腺样囊性癌中的MYB和核因子I/B(NF1B)重排,肌上皮瘤和肌上皮癌中的尤文肉瘤断点区域1(EWSR1)位点或FUS(TLS)位点重排以及玻璃样透明细胞癌中的EWSR1和激活转录因子1(ATF1)重排。PSGT整体预后较好,治疗原则与非小细胞肺癌类似。近年来,对于PSGT各亚型的诊断和治疗虽取得一些进展,但仍然存在很多复杂且具有挑战性的问题。文章就PSGT的诊断和治疗进行综述。Primary salivary gland-type tumors of the lung(PSGT)originating from the submucosal glands of the major airways,are rare pulmonary malignancies,accounting for less than 0.2%of all primary lung tumors.The diagnosis of PSGT requires differentiation from other primary lung tumors and metastatic diseases from extra-pulmonary sources,especially the salivary glands in the head and neck.Molecular alterations play a pivotal role in the diagnosis of PSGT,such as the CRTCl::MAML2 rearrangement in mucoepidermoid carcinoma,the MYB:NF1B rearrangement in adenoid cystic carcinoma,the rearrangement of the EWSR1 locus or the FUS(TLS)locus in myoepithelioma and myoepithelial carcinoma,and the EWSRl::ATF1 rearrangement in hyaline cell carcinoma.The prognosis of PSGT is good,and the treatment principles are similar to those of nonsmall cell lung cancer.Recent years,although some progress has been made in the diagnosis and treatment of each subtype of PSGT,there are still many complex and challenging issues.This article provides a review of the diagnosis and treatment of primary salivary gland-type tumors of the lung.

关 键 词：肺肿瘤 涎腺型肿瘤 黏液表皮样癌 腺样囊性癌 

分 类 号：R734.2[医药卫生—肿瘤]