Oncotarget

Reviews:

Advances in sarcoma diagnostics and treatment

Amanda R. Dancsok _, Karama Asleh-Aburaya and Torsten O. Nielsen

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Oncotarget. 2017; 8:7068-7093. https://doi.org/10.18632/oncotarget.12548

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Abstract

Amanda R. Dancsok1, Karama Asleh-Aburaya1 and Torsten O. Nielsen1,2

1 Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada

2 Sarcoma Disease Site Committee, Canadian Cancer Trials Group

Correspondence to:

Amanda R. Dancsok, email:

Keywords: soft tissue sarcoma, sarcoma review, sarcoma diagnostics, sarcoma therapeutics, sarcoma advances

Received: July 25, 2016 Accepted: September 29, 2016 Published: October 09, 2016

Abstract

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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