title: Advances in sarcoma diagnostics and treatment.
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.
authors.affiliation: Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.
authors.forename: Amanda R
authors.initials: AR
authors.lastname: Dancsok
authors.affiliation: Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.
authors.forename: Karama
authors.initials: K
authors.lastname: Asleh-Aburaya
authors.affiliation: Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.|Sarcoma Disease Site Committee, Canadian Cancer Trials Group.
authors.forename: Torsten O
authors.initials: TO
authors.lastname: Nielsen
mesh_terms: D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D002986:Clinical Trials as Topic; D019468:Disease Management; D044127:Epigenesis, Genetic / Q000187:drug effects; D020869:Gene Expression Profiling; D053263:Gene Regulatory Networks / Q000187:drug effects; D018740:Genetic Heterogeneity; D059014:High-Throughput Nucleotide Sequencing; D006801:Humans; D058990:Molecular Targeted Therapy; D012509:Sarcoma / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics
chemical_list: D000970:Antineoplastic Agents
keywords: sarcoma advances; sarcoma diagnostics; sarcoma review; sarcoma therapeutics; soft tissue sarcoma