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Review
. 2019 Aug;43(4):300-307.
doi: 10.1016/j.currproblcancer.2019.06.004. Epub 2019 Jun 20.

Future directions in soft tissue sarcoma treatment

Francis Hall  1 , Victor Villalobos  2 , Breelyn Wilky  2
Affiliations
Review

Future directions in soft tissue sarcoma treatment

Francis Hall et al. Curr Probl Cancer. 2019 Aug.

Abstract

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 PDGFRα/KIT, β-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.

Keywords: ALK fusion; ASPSCR1-TFE3 fusion; EZH2/INI1; IDH mutation; Immunotherapy; KIT; MDM2; PDGFRα; Sarcoma; Soft tissue sarcoma; Targeted therapy; β-catenin/APC/NOTCH.

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