Future directions in soft tissue sarcoma treatment

Francis Hall; Victor Villalobos; Breelyn Wilky

doi:10.1016/j.currproblcancer.2019.06.004

Future directions in soft tissue sarcoma treatment

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

Authors

Francis Hall¹, Victor Villalobos², Breelyn Wilky²

Affiliations

¹ Department of Internal Medicine, Division of Medical Oncology, University of Colorado Denver Anschutz Medical Campus, Aurora, Colorado. Electronic address: francis.hall@ucdenver.edu.
² Department of Internal Medicine, Division of Medical Oncology, University of Colorado Denver Anschutz Medical Campus, Aurora, Colorado.

PMID: 31229264
DOI: 10.1016/j.currproblcancer.2019.06.004

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.

Publication types

Review

MeSH terms

Combined Modality Therapy
Disease Management
Humans
Immunotherapy / methods*
Molecular Targeted Therapy / methods*
Prognosis
Sarcoma / pathology
Sarcoma / therapy*