Journal
ONCOTARGET
Volume 8, Issue 4, Pages 7068-7093Publisher
IMPACT JOURNALS LLC
DOI: 10.18632/oncotarget.12548
Keywords
soft tissue sarcoma; sarcoma review; sarcoma diagnostics; sarcoma therapeutics; sarcoma advances
Categories
Funding
- Canadian Cancer Society Research Institute [701582]
- Terry Fox Research Institute (TFF) [105265]
- Liddy Shriver Sarcoma Initiative (ImmunoSarc)
- Sarcoma Cancer Foundation of Canada (Beth England's Sarcoma Research Fund)
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The heterogeneity of sarcomas with regard to molecular genesis, histology, clinical characteristics, and response to treatment makes management of these rare yet diverse neoplasms particularly challenging. This review encompasses recent developments in sarcoma diagnostics and treatment, including cytotoxic, targeted, epigenetic, and immune therapy agents. In the past year, groups internationally explored the impact of adding mandatory molecular testing to histological diagnosis, reporting some changes in diagnosis and/or management; however, the impact on outcomes could not be adequately assessed. Transcriptome sequencing techniques have brought forward new diagnostic tools for identifying fusions and/or characterizing unclassified entities. Next-generation sequencing and advanced molecular techniques were also applied to identify potential targets for directed and epigenetic therapy, where preclinical studies reported results for agents active within the receptor tyrosine kinase, mTOR, Notch, Wnt, Hedgehog, Hsp90, and MDM2 signaling networks. At the level of clinical practice, modest developments were seen for some sarcoma subtypes in conventional chemotherapy and in therapies targeting the pathways activated by various receptor tyrosine kinases. In the burgeoning field of immune therapy, sarcoma work is in its infancy; however, elaborate protocols for immune stimulation are being explored, and checkpoint blockade agents advance from preclinical models to clinical studies.
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