Targeted therapy for malignant peripheral nerve sheath tumor: translational research and clinical application
10.3760/cma.j.issn.0253?3766.2019.09.002
- VernacularTitle:恶性周围神经鞘瘤靶向治疗的研究进展
- Author:
Zhichao LIAO
1
;
Chao ZHANG
;
Xinyue LIU
;
Zhiwu REN
;
Jin XU
;
Chunzhi ZHANG
;
Yun YANG
;
Ze ZHU
;
Jilong YANG
Author Information
1. 天津医科大学肿瘤医院骨与软组织肿瘤科 国家肿瘤临床医学研究中心 天津市"肿瘤防治"重点实验室 天津市恶性肿瘤临床医学研究中心 300060
- Keywords:
Malignant peripheral nerve sheath tumor;
Targeted therapy;
Translational research;
Clinical trial
- From:
Chinese Journal of Oncology
2019;41(9):648-653
- CountryChina
- Language:Chinese
-
Abstract:
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.
