(Nationwide Children’s Hospital) 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 translocation t(11;22)(q24;q12), which creates the fusion protein and oncogenic driver EWS/FLI.

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Abstract
Ewing sarcoma was first described in 1921 in the Proceedings of the New York Pathological Society by an eminent American pathologist from Cornell named James R. Ewing as a “diffuse endothelioma of bone.” Since this initial description, more has been discovered regarding Ewing sarcoma and in the 1980’s both Ewing sarcoma and peripheral primitive neuroectodermal tumors due to their similar features and shared identical genetic abnormality were grouped into a class of cancers entitled Ewing sarcoma family of tumors (ESFTs). Ewing sarcoma is the second most common pediatric osseous malignancy follow…

Abstract
Desmoplastic Small Round Cell Tumor (DSRCT) is a rare sarcoma tumor of adolescence and young adulthood, which harbors a recurrent chromosomal translocation between the Ewing’s sarcoma gene (EWSR1) and the Wilms’ tumor suppressor gene (WT1). Patients usually develop multiple abdominal tumors with liver and lymph node metastasis developing later. Survival is poor using a multimodal therapy that includes chemotherapy, radiation and surgical resection, new therapies are needed for better management of DSRCT. Triggering cell apoptosis is the scientific rationale of many cancer therapies. Here, we characterized…

Source: NeoplasiaCategory: Cancer & Oncology Authors: Tags: Neoplasia Source Type: research

Conclusion: FISH is a useful adjunct in the
diagnostic assessment of different types of soft tissue sarcomas. It is easy to set up, is relatively inexpensive and has
the ability to diagnose sarcomas with great accuracy, especially in cases which can not be accurately classified even
after thorough histological and immunohistochemical evaluation. It may play a very important role in the accurate
diagnosis and correct management of patients.
PMID: 29580035 [PubMed – in process]

EWS–FLI1 increases transcription to cause R-loops and block BRCA1 repair in Ewing sarcoma

Nature 555, 7696 (2018). doi:10.1038/nature25748

Authors: Aparna Gorthi, July Carolina Romero, Eva Loranc, Lin Cao, Liesl A. Lawrence, Elicia Goodale, Amanda Balboni Iniguez, Xavier Bernard, V. Pragathi Masamsetti, Sydney Roston, Elizabeth R. Lawlor, Jeffrey A. Toretsky, Kimberly Stegmaier, Stephen L. Lessnick, Yidong Chen &Alexander J. R. Bishop
Ewing sarcoma is an aggressive paediatric cancer of the bone and soft tissue. It results from a chromosomal translocation, predominantly t(11;22)(q24:q12), that fus…

Source: NatureCategory: Research Authors: Tags: Letter Source Type: research

Authors: Bulbul A, Fahy BN, Xiu J, Rashad S, Mustafa A, Husain H, Hayes-Jordan A
Abstract
Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17 to 25 months, and 5-year survival rates remain around 15%, with higher survival reported among those undergoing removal of at least 90…

Source: SarcomaCategory: Cancer & Oncology Tags: Sarcoma Source Type: research

Ewing sarcoma is a bone malignancy driven by a translocation event resulting in the fusion protein EWS/FLI1 (EF). EF functions as an aberrant and oncogenic transcription factor that misregulates the expression of thousands of genes. Previous work has focused principally on determining important transcriptional targets of EF, as well as characterizing important regulatory partnerships in EF-dependent transcriptional programs. Less is known, however, about EF-dependent metabolic changes or their role in Ewing sarcoma biology. Therefore, the metabolic effects of silencing EF in Ewing sarcoma cells were determined. Metabolomic…

Source: Molecular Cancer ResearchCategory: Cancer & Oncology Authors: Tags: Metabolism Source Type: research

tsky JA
Abstract
Ewing’s sarcoma (ES) is a rare and highly malignant cancer that grows in the bones or surrounding tissues mostly affecting adolescents and young adults. A chimeric fusion between the RNA binding protein EWS and the ETS family transcription factor FLI1 (EWS-FLI1), which is generated from a chromosomal translocation, is implicated in driving most ES cases by modulation of transcription and alternative splicing. The small-molecule YK-4-279 inhibits EWS-FLI1 function and induces apoptosis in ES cells. We aimed to identify both the underlying mechanism of the drug and potential combination therapies th…

Source: Science SignalingCategory: Biomedical Science Authors: Tags: Sci Signal Source Type: research

Ewing’s sarcoma (ES) is a rare and highly malignant cancer that grows in the bones or surrounding tissues mostly affecting adolescents and young adults. A chimeric fusion between the RNA binding protein EWS and the ETS family transcription factor FLI1 (EWS-FLI1), which is generated from a chromosomal translocation, is implicated in driving most ES cases by modulation of transcription and alternative splicing. The small-molecule YK-4-279 inhibits EWS-FLI1 function and induces apoptosis in ES cells. We aimed to identify both the underlying mechanism of the drug and potential combination therapies that might enhance its…

Source: Signal Transduction Knowledge EnvironmentCategory: Science Authors: Tags: STKE Research Articles Source Type: news

This report highlights the importance of monitoring for treatment-related effects in cancer therapy, as well as the need for novel, less toxic approaches in Ewing sarcoma therapy.

Contributors : Stefan K Z öllner ; Saravana P Selvanathan ; Garrett T Graham ; Ryan M Commins ; Sung-Hyeok Hong ; Eric Moseley ; Sydney Parks ; Jessica N Haladyna ; Hayriye V Erkizan ; Uta Dirksen ; Michael D Hogarty ; Aykut Üren ; Jeffrey A ToretskySeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensA chimeric fusion between the RNA binding protein EWS and the ETS family transcription factor FLI1 (EWS-FLI1), created from a chromosomal translocation, is implicated in driving the majority of Ewing sarcomas (ES) by modulation of transcription and alternative splicing. The small mo…

Source: GEO: Gene Expression OmnibusCategory: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research





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