摘要
恶性周围神经鞘瘤(MPNST)是一种少见的起源于外周神经分支和神经鞘膜的侵袭性软组织肉瘤。早期根治性手术是MPNST有效的治疗方式,但由于MPNST对放疗和化疗的敏感性低,疾病进展迅速,预后通常较差,死亡率高。近年来,寻找MPNST演进的驱动因素及治疗靶点的转化研究和临床试验发展迅速,涉及NF1-Ras途径、Raf-MEK-ERK途径、PI3K-AKT-mTOR途径、Wnt信号通路、凋亡蛋白的异常表达、多梳抑制复合物2普遍缺失、HDAC家族高表达、受体酪氨酸激酶异常、细胞程序性死亡配体表达、极光激酶表达以及多种微小RNA等方面。文章归纳了与MPNST相关的潜在治疗靶点的转化研究,并对正在研发的靶向药物及其临床试验进行总结,为MPNST靶向治疗的临床应用提供有效信息。
Malignant peripheral nerve sheath tumor (MPNST) is a rare invasive soft tissue sarcoma that originates from peripheral nerve branches and peripheral nerve sheaths. Early radical surgery is an effective treatment for MPNST. Since it is insensitive to radiotherapy and chemotherapy, the disease manifests a rapid progression, poor prognosis and high mortality. In recent years, the translational researches on the driving factors and therapeutic targets of MPNST have been rapidly developed, including the pathways of NF1-Ras, Raf-MEK-ERK, PI3K-AKT-mTOR, Wnt signaling, and abnormal expressions of apoptotic proteins, the general loss of polycomb repressive complex 2 (PRC2), upregulation of the HDAC family, abnormal expressions of receptor tyrosine kinases, expressions of programmed cell death ligand (PD-L1), aurora kinase and various microRNAs.This review summarizes the current translational researches on potential therapeutic targets of MPNST, and the clinical trials which provide helpful information for MPNST targeted therapy.
作者
廖智超
张超
刘新月
任志午
徐进
张春智
杨蕴
朱泽
杨吉龙
Liao Zhichao;Zhang Chao;Liu Xinyue;Ren Zhiwu;Xu Jin;Zhang Chunzhi;Yang Yun;Zhu Ze;Yang Jilong(Department of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center of Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin′s Clinical Research Center for Cancer, Tianjin 300060, China;Department of Anesthesiology, Tianjin Hospital, Tianjin 300211, China;Department of Radiotherapy, Tianjin Hospital, Tianjin 300211, China;School of Basic Medical Sciences, Tianjin Medical University, Tianjin 300070, China)
出处
《中华肿瘤杂志》
CAS
CSCD
北大核心
2019年第9期648-653,共6页
Chinese Journal of Oncology
基金
天津市重点研发计划科技支撑重点项目(18YFZCSY00550)
天津自然科学基金(16JCYBJC24100).
关键词
恶性周围神经鞘瘤
靶向治疗
转化研究
临床试验
Malignant peripheral nerve sheath tumor
Targeted therapy
Translational research
Clinical trial