Sarcoma Diagnosis and Treatment

High Tau Expression Correlates with Reduced Invasion and Prolonged Survival in Ewing Sarcoma

This study finds that high Tau expression in Ewing sarcoma cells reduces invasion and improves patient survival.

Differential cell-ECM interaction of rhabdomyosarcoma subtypes regulated by PAX3-FOXO1

This study identifies differences in cell-ECM interaction between rhabdomyosarcoma subtypes, showing FNRMS has heightened interaction regulated by TGFβ signaling, while PAX3-FOXO1 in FPRMS suppresses this interaction.

Dynamic modelling of EWS::FLI1 fluctuations reveals molecular determinants of phenotypic tumor plasticity and prognosis in Ewing sarcoma

This study models EWS::FLI1 fluctuations in Ewing sarcoma, showing that partial depletion promotes metastasis and identifies gene clusters and transcription factors linked to poor prognosis.

Spatial profiling identifies regionally distinct microenvironments and targetable immunosuppressive mechanisms in pediatric osteosarcoma pulmonary metastases.

Visium profiling of nine pediatric osteosarcoma pulmonary metastases reveals a conserved granuloma-like architecture: inflamed macrophage-rich periphery, collagen capsule, inflammation-poor core, and CXCR4 as candidate target.

Super-enhancer-driven CACNA2D2 is an EWSR1::WT1 signature gene encoding a diagnostic marker for desmoplastic small round cell tumor (DSRCT)

The paper identifies CACNA2D2 as a super-enhancer-driven target of EWSR1::WT1 and demonstrates its utility as a diagnostic biomarker for DSRCT.

Rewiring the fusion oncoprotein EWS/FLI1 in Ewing sarcoma with bivalent small molecules

This study develops a bivalent small molecule, EB-TCIP, that relocalizes EWS/FLI1 to induce BCL6 target genes, demonstrating a strategy to target fusion oncoproteins in Ewing sarcoma.

YAP1 is a key regulator of EWS::FLI1-dependent malignant transformation upon IGF-1 mediated reprogramming of bone mesenchymal stem cells

The paper shows that IGF-1 reprograms bone mesenchymal stem cells in EWS::FLI1-mutant mice, leading to tumor formation, with Yap1 playing a central role, and suggests combined IGF-1R and YAP/TEAD inhibition as a potential treatment.

Physiologically refined cell culture conditions uncover oncogene-dependent metabolic signatures in Ewing sarcoma spheroids

This study improves Ewing sarcoma cell culture conditions to better reflect physiological states, revealing metabolic gene signatures linked to oncogenic fusion proteins.

Tumor-associated stroma shapes the spatial tumor immune microenvironment of primary Ewing sarcomas

This study uses spatial transcriptomics to analyze Ewing sarcoma tumors, finding that localized tumors have more stromal enrichment and unique immune signals compared to metastatic tumors.

Synthetic inhibition of the SUMO pathway by targeting the SAE1 component via TAK-981 compound impairs tumor growth and chemosensitizers embryonal and alveolar rhabdomyosarcoma cell lines.

This study shows that TAK-981, a SUMO inhibitor targeting SAE1, reduces RMS cell growth and enhances chemotherapy effectiveness without increasing radiosensitivity.

EWS-FLI1 Expression in Human Embryonic MSCs Leads to Transcriptional Reprograming, Defective DNA Damage Repair and Ewing Sarcoma

This study shows that EWS-FLI1 expression in human embryonic mesenchymal stem cells leads to an ES-like transcriptome and tumor formation, despite not preferentially binding promoters.

Inhibition of DKK-1 by WAY262611 Inhibits Osteosarcoma Metastasis

This study shows that the small molecule inhibitor WAY262611 inhibits osteosarcoma metastasis by activating Wnt signaling and inducing osteoblastic differentiation.

Genetic and Epigenetic Characterization of Sarcoma Stem Cells Across Subtypes Identifies EZH2 as a Therapeutic Target

The paper identifies EZH2 as a therapeutic target in sarcoma stem cells (STS-CSCs) across subtypes, showing that targeting EZH2 reduces CSC populations and restores chemosensitivity in resistant cell lines.

Transcriptomic Profiling of Old Age Sarcoma Patients using TCGA RNA-seq data

This study analyzes TCGA-SARC RNA-seq data to compare transcriptomic profiles between older and younger sarcoma patients, identifying differentially expressed genes and pathways associated with poor prognosis in older patients.

Targeting Osteosarcoma heterogeneity to improve therapeutic response

The paper identifies transcription factors that drive osteosarcoma cell states and shows that modulating them can increase sensitivity to targeted therapy.

Analysis of publicly available transcriptomic data to identify key genes and pathways associated with osteosarcoma metastasis

This study analyzes 8 publicly available transcriptomic datasets to identify common deregulated genes and pathways associated with osteosarcoma onset and metastasis.

The Capicua C1 Domain is Required for Full Activity of the CIC::DUX4 Fusion Oncoprotein

This study shows that the C1 domain of CIC is required for full activity of the CIC::DUX4 fusion oncoprotein, highlighting the importance of breakpoint location in fusion function.

Endogenous EWSR1-FLI1 degron alleles enable control of fusion oncoprotein expression in tumor cell lines and xenografts

This study creates inducible EWSR1-FLI1 degron alleles in tumor cell lines to control fusion oncoprotein expression, showing reversible cell cycle arrest and tumor growth suppression in xenografts.

Integrated Molecular Profiling of Rhabdomyosarcoma Subtypes by Targeted RNAseq

This paper develops a targeted RNAseq assay to simultaneously detect multiple genetic alterations in rhabdomyosarcoma subtypes, improving diagnostic efficiency and accuracy.

Gli2 Overexpression Alters the Differentiation Status of Dedifferentiated Liposarcoma Cells and Results in an Immunosuppressive Myeloid Phenotype in Orthotopic Tumors

This study shows that Gli2 overexpression in dedifferentiated liposarcoma cells reduces adipogenic differentiation, increases osteoblast markers, and promotes an immunosuppressive myeloid phenotype in tumors.

A Self-Assembling Immune-Featured Osteosarcoma Patient/PDX Derived Organoid Model and Biobank for Personalized Immune Therapy

The paper develops a patient/PDX-derived osteosarcoma organoid model and biobank to enable rapid testing of personalized immune therapies and drug development.

PAX fusion proteins deregulate gene networks controlling mitochondrial translation in pediatric rhabdomyosarcoma

This study identifies PAX3-FOXO1 and PAX7-FOXO1 fusion proteins as drivers of mitochondrial translation gene networks in pediatric rhabdomyosarcoma, offering new therapeutic targets.

The variable genomic landscape during osteosarcoma progression: insights from a longitudinal WGS analysis

This study analyzes the genomic changes in osteosarcoma over time using WGS, revealing increasing genomic complexity and variable evolutionary patterns.

A dynamic microRNA profile that tracks a chemotherapy resistance phenotype in osteosarcoma. Implications for novel therapeutics.

The paper identifies a dynamic microRNA profile associated with chemotherapy resistance in osteosarcoma and predicts novel therapeutic targets that could reverse resistance.

BCOR-Rearranged Sarcomas: In Silico Insights into Altered Domains and Reduced RAWUL-PUFD Binding

The paper investigates how BCOR fusion proteins alter domain structure and RAWUL-PUFD binding, impacting PRC1 function and gene regulation in BRS.

Seclidemstat blocks the transcriptional function of multiple FET-fusion oncoproteins

This study shows seclidemstat inhibits the transcriptional activity of multiple FET fusion oncoproteins in vitro, suggesting it as a promising treatment for FET-rearranged sarcomas.

Immunocompetent murine model of Ewing sarcoma reveals role for TGFβ inhibition to enhance immune infiltrates in Ewing tumors during radiation

The paper develops an immunocompetent mouse model of Ewing sarcoma to show that TGFβ inhibition enhances immune infiltration and reduces metastasis during radiation therapy.

Glutaredoxin 3 (GLRX3) confers a fusion oncogene-dependent vulnerability to Ewing sarcoma

This study identifies GLRX3 as a vulnerability in Ewing sarcoma driven by EWSR1::FLI1 fusion oncoproteins, showing its role in oxidative stress and iron homeostasis and potential therapeutic targeting.

An EWS::FLI1 monoclonal antibody with multimodal utility and specificity for FLI1

The paper develops a monoclonal antibody that specifically recognizes EWS::FLI1 for use in molecular biology applications.

Systematic multi-omics cell line profiling uncovers principles of Ewing sarcoma fusion oncogene-mediated gene regulation

The paper presents a multi-omics cell line atlas for Ewing sarcoma, identifying new target genes and regulatory mechanisms of EWSR1-ETS fusion oncogenes.