The FLI Portion of EWS/FLI Contributes a Transcriptional Regulatory Function That Is Distinct and Separable from Its DNA-Binding Function in Ewing Sarcoma

Investigators sought to define the specific contributions of each FLI domain to EWS/FLI activity in a well-validated Ewing sarcoma model and, in doing so, to better understand Ewing sarcoma development mediated by the fusion protein.
[Oncogene]
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Salarius Pharmaceuticals Announces New Clinical Trial to Study Seclidemstat in Hematologic Cancers

Salarius Pharmaceuticals, Inc. announced the initiation of a clinical trial to investigate seclidemstat, a novel lysine specific demethylase 1 inhibitor, as a potential treatment for hematologic cancers. This investigator-initiated Phase I/II trial will be led by Dr. Guillermo Montalban-Bravo from the The University of Texas MD Anderson Cancer Center.
[Salarius Pharmaceuticals, Inc.]
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Integrated Diagnosis Based on Transcriptome Analysis in Suspected Pediatric Sarcomas

To evaluate the potential utility of including RNA-sequencing in the diagnostic process, investigators performed RNA-sequencing in 47 patients with suspected pediatric sarcomas.
[npj Genomic Medicine]
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STAG2 Loss Rewires Oncogenic and Developmental Programs to Promote Metastasis in Ewing Sarcoma

The core cohesin subunit STAG2 is recurrently mutated in Ewing sarcoma but its biological role is less clear. The authors demonstrated that cohesin complexes containing STAG2 occupy enhancer and polycomb repressive complex-marked regulatory regions.
[Cancer Cell]
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SPOP and OTUD7A Control EWS–FLI1 Protein Stability to Govern Ewing Sarcoma Growth

Speckle-type POZ protein (SPOP) and OTU domain-containing protein 7A (OTUD7A) are identified as the bona fide E3 ligase and deubiquitinase, respectively, that control Ewing sarcoma breakpoint region 1-Friend leukemia integration 1 (EWS–FLI1) protein turnover in Ewing sarcoma.
[Advanced Science]
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Moleculin Commences Phase Ib/II Clinical Trial of Annamycin for the Treatment of Sarcoma Lung Metastases

Moleculin Biotech, Inc. announced it has received clearance to initiate its Phase Ib/II clinical trial evaluating Annamycin for the treatment of soft tissue sarcoma lung metastases.
[Moleculin Biotech, Inc.]
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Phase II Study of Pazopanib with Oral Topotecan in Patients with Metastatic and Non-Resectable Soft Tissue and Bone Sarcomas

This prospective, single arm, Phase II study evaluated the efficacy of the combination of pazopanib with topotecan in patients with metastatic or unresectable non-adipocytic soft-tissue sarcoma
[British Journal of Cancer]
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TP53 Mutations Increase Radioresistance in Rhabdomyosarcoma and Ewing Sarcoma

Investigators determined the clinical phenotype of TP53 mutations and p53 pathway alterations in patients with rhabdomyosarcoma and Ewing sarcoma treated with radiation.
[British Journal of Cancer]
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ETV4/5 Drive Synovial Sarcoma through Control of the Cell Cycle and the DUX4 Embryonic Pathway

In genetic FGFR knockout models, culture, and xenograft synovial sarcoma models treated with the FGFR inhibitor BGJ398, researchers showed that FGFR1, FGFR2, and FGFR3 were crucial for tumor growth.
[Journal of Clinical Investigation]
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Noxopharm’s Veyonda to Begin First-Line Sarcoma Treatment Testing

Noxopharm Limited announced its CEP-2 study will begin shortly. CEP-2 is related to the Investigational New Drug approval of Veyonda® by the FDA based on evidence that Veyonda may increase generally poor response rates of sarcoma cancers to chemotherapy in combination with common chemotherapy drug, doxorubicin, for patients with soft tissue sarcomas.
[Noxopharm Limited]
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Germinal GLT8D1, GATAD2A and SLC25A39 Mutations in a Patient with a Glomangiopericytal Tumor and Five Different Sarcomas over a 10-Year Period

The authors reported the unique case of a 72-year-old patient who presented with glomangiopericytal tumor followed by six sarcomas of the extremities between 2007 and 2016.
[Scientific Reports]
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