Review
Targeted therapy for malignant peripheral nerve sheath tumor: translational research and clinical application
Liao Zhichao, Zhang Chao, Liu Xinyue, Ren Zhiwu, Xu Jin, Zhang Chunzhi, Yang Yun, Zhu Ze, Yang Jilong
Published 2019-09-23
Cite as Chin J Oncol, 2019, 41(9): 648-653. DOI: 10.3760/cma.j.issn.0253-3766.2019.09.002
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.
Key words:
Malignant peripheral nerve sheath tumor; Targeted therapy; Translational research; Clinical trial
Contributor Information
Liao Zhichao
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
Zhang Chao
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
Liu Xinyue
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
Ren Zhiwu
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
Xu Jin
Department of Anesthesiology, Tianjin Hospital, Tianjin 300211, China
Zhang Chunzhi
Department of Radiotherapy, Tianjin Hospital, Tianjin 300211, China
Yang Yun
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
Zhu Ze
School of Basic Medical Sciences, Tianjin Medical University, Tianjin 300070, China
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
