Ewing Sarcoma

New Guidelines Offer a Clear Path for Expandable Endoprosthesis Use in Post-Sarcoma Limb Reconstruction
New Guidelines Offer a Clear Path for Expandable Endoprosthesis Use in Post-Sarcoma Limb Reconstruction 150 150 Abbie Miller

Limb salvage operation is the standard of care for pediatric extremity bone sarcomas. These tumors often occur during the second decade of life, when children are growing rapidly. Reconstructive surgeries must account for anticipated remaining growth and the potential for resulting limb inequity. Osteosarcoma and Ewing sarcoma are the most common primary malignant bone tumors,…

Ewing Sarcoma Transcription Factor Mediates Changes in Transcription by Reprogramming Chromatin Structure
Ewing Sarcoma Transcription Factor Mediates Changes in Transcription by Reprogramming Chromatin Structure 1024 575 Abbie Miller
yellow squiggly lines representing chromatin strands in the nucleus

By binding to DNA in the nucleus, the Ewing sarcoma-specific transcription factor EWS/FLI changes chromatin organization, leading to altered gene expression and the development of Ewing sarcoma.   A new report, published in Nucleic Acids Research, focuses on how the Ewing sarcoma-specific transcription factor EWS/FLI changes spatial organization of DNA in the nucleus of Ewing…

Distinct Transcriptional Regulatory Domain Identified in Ewing Sarcoma Fusion Protein
Distinct Transcriptional Regulatory Domain Identified in Ewing Sarcoma Fusion Protein 1024 1024 Lauren Dembeck
Illustration of split DNA - Single strand ribonucleic acid

A better understanding of a newly defined region in the fusion protein that causes Ewing sarcoma may lead to novel approaches for therapeutic targeting. Ewing sarcoma is an aggressive pediatric bone cancer defined by the presence of a single genetic abnormality: a chromosomal translocation. The translocation splits two genes and joins them abnormally, creating a…

A Novel FUS/ETV4 Fusion and Other Rare FET/ETS Fusion Proteins Are Bona Fide Ewing Sarcoma
A Novel FUS/ETV4 Fusion and Other Rare FET/ETS Fusion Proteins Are Bona Fide Ewing Sarcoma 1024 575 Jessica Nye, PhD

Researchers conduct a molecular comparison between the established EWS/FLI translocation with rarer FET/ETS fusions, broadly supporting the inclusion of FET/ETS chimeric proteins in Ewing Sarcoma diagnosis and treatment.   Ewing sarcoma, most commonly caused by a chromosomal translocation of the amino-terminal domain of EWSR1 with the transcription factor FLI (EWS/FLI), presents as an aggressive pediatric…

Two Classes of GGAA-Microsatellites in a Ewing Sarcoma Context
Two Classes of GGAA-Microsatellites in a Ewing Sarcoma Context 150 150 Abbie Miller

Characterization of GGAA-microsatellites provides insight in the role of noncoding DNA in cancer susceptibility and therapeutic development. In a study published in PLOS ONE, researchers describe two types of GGAA-microsatellites and their roles in EWS/FLI binding and gene regulation in Ewing sarcoma. Ewing sarcoma is the second most common pediatric bone malignancy. It is initiated by chromosomal…

A New Paradigm for Treating Transcription Factor-Driven Cancers
A New Paradigm for Treating Transcription Factor-Driven Cancers 150 150 Abbie Miller

Researchers describe how the association of the EWS/FLI transcription factor with GGAA-microsatellites drives Ewing sarcoma. In the current issue of Proceedings of the National Academy of Sciences, researchers from Nationwide Children’s Hospital describe a new paradigm for treating transcription factor-driven cancers. The study focuses on Ewing sarcoma and how the EWS/FLI transcription factor drives the malignancy…