期刊
CURRENT PROBLEMS IN CANCER
卷 43, 期 4, 页码 300-307出版社
MOSBY-ELSEVIER
DOI: 10.1016/j.currproblcancer.2019.06.004
关键词
Sarcoma; Soft tissue sarcoma; Targeted therapy; Immunotherapy; PDGFR alpha; KIT; beta-catenin/APC/NOTCH; IDH mutation; MDM2; EZH2/INI1; ALK fusion; ASPSCR1-TFE3 fusion
类别
Sarcoma is a broad term for mesenchymal malignancies that arise from soft tissue or bone. Despite classification by histologic subtype, clinical behavior and response to therapy have great variability. Modern genetic sequencing techniques have been able to identify additional genetic variability and subsequently new targeted therapies. In this review, we discuss the current state of STS diagnostics and treatment and explore some of the more promising areas in which progress is being made. We discuss therapies targeting PDGFR alpha/KIT, beta-Catenin/APC/NOTCH, IDH-1/2 mutations, MDM2 amplifications, EZH2/INI1 expression loss, ALK fusion, and ASPSCR1-TFE3 fusion. We also discuss the progress that has been made within immunotherapies. While soft tissue sarcomas still portend a poor prognosis, these targeted therapies and immunotherapies provide treatment with less toxic side effects. (C) 2019 Elsevier Inc. All rights reserved.
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