Ewing sarcoma

@article{Grnewald2018EwingS,
  title={Ewing sarcoma},
  author={Thomas G. P. Gr{\"u}newald and Florencia Cidre-Aranaz and Didier Surdez and Eleni M. Tomazou and Enrique de {\'A}lava and Heinrich Kovar and Poul H. Sorensen and Olivier Delattre and Uta Dirksen},
  journal={Nature Reviews Disease Primers},
  year={2018},
  volume={4},
  pages={1-22}
}
Ewing sarcoma is the second most frequent bone tumour of childhood and adolescence that can also arise in soft tissue. Ewing sarcoma is a highly aggressive cancer, with a survival of 70–80% for patients with standard-risk and localized disease and ~30% for those with metastatic disease. Treatment comprises local surgery, radiotherapy and polychemotherapy, which are associated with acute and chronic adverse effects that may compromise quality of life in survivors. Histologically, Ewing sarcomas… 
Systems Biology Analysis for Ewing Sarcoma.
TLDR
It is concluded that despite the seeming simplicity of the oncogene action of EwS, a lot has yet to be understood on the systems-wide mechanisms connecting the driver mutation and the major cellular phenotypes of this pediatric cancer.
EWSR1-NFATC2 and FUS-NFATC2 Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review
TLDR
Three patients with tumors carrying the EWSR1-NFATC2 gene translocation are reported, including one rare primary tumor of soft tissues and another patient with a benign-appearing bone tumor with a unique FUS-NFatC2 genes translocation is described.
Molecular Approaches to Diagnosis in Ewing Sarcoma: RT-PCR.
TLDR
A RT-PCR method is described that allows for the detection of the most frequent alterations with elevated specificity and sensitivity which is able to distinguish among the different types of fusions in Ewing sarcoma.
Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives
TLDR
This review provides scientific answers to clinical phenomena and occasionally defines the resulting translational studies needed to overcome the hurdle of treatment-associated morbidities and, most importantly, non-survival in patients with Ewing sarcoma.
Gene expression and immunohistochemical analyses identify SOX2 as major risk factor for overall survival and relapse in Ewing sarcoma patients
TLDR
High SOX2 expression constitutes an independent prognostic biomarker for EwS patients with poor outcomes and may help to identify patients with localised disease who are at high risk for tumour relapse within the first two years after diagnosis.
Targeting the undruggable: exploiting neomorphic features of fusion oncoproteins in childhood sarcomas for innovative therapies
TLDR
A broad overview of the current literature on targeting neomorphic features of fusion oncogenes found in Ewing sarcoma, alveolar rhabdomyosarcomas, and synovial sarcomas is provided, and a perspective for future developments is given.
Extracellular Vesicles in Reprogramming of the Ewing Sarcoma Tumor Microenvironment
TLDR
Ewing sarcoma EVs composition, their potential role in metastasis and in the reprogramming of various cells of the tumor microenvironment, and the potential for clinical intervention are detailed.
Overexpression of EWSR1 (Ewing sarcoma breakpoint region 1/EWS RNA binding protein 1) predicts poor survival in patients with hepatocellular carcinoma.
TLDR
Univariate and multivariate Cox hazard regression analysis results revealed that EWSR1 was an independent prognostic factor for OS in HCC, and bioinformatics analysis showed RNA splicing process represented the major function and pathway.
Breakthrough Technologies Reshape the Ewing Sarcoma Molecular Landscape
TLDR
Next-generation technologies applied to liquid biopsies will significantly impact patient management by allowing the early detection of relapse and monitoring response to treatment, and the use of these novel technologies has provided data of great value in order to discover new druggable pathways.
Treatment-driven selection of chemoresistant Ewing sarcoma tumors with limited drug distribution.
TLDR
In mice receiving irinotecan infusions, the level of SN-38 in the intracellular tumor compartment was reduced in tumors at later stages compared to earlier tumors for those pairs bearing similar CNAs, suggesting that distribution of anticancer drug shifted toward the extracellular compartment during clonal tumor evolution.
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References

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EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma
TLDR
This work describes a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance and discusses their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development.
Development of Ewing's sarcoma from primary bone marrow-derived mesenchymal progenitor cells.
TLDR
It is shown that EWS-FLI-1 alone can transform primary bone marrow-derived mesenchymal progenitor cells and generate tumors that display hallmarks of Ewing's sarcoma, including a small round cell phenotype, expression of EFT-associated markers, insulin like growth factor-I dependence, and induction or repression of numerous E WS-FLi-1 target genes.
The genomic landscape of pediatric Ewing sarcoma.
TLDR
Next-generation sequencing of Ewing sarcoma found remarkably few mutations, however, it was discovered that loss of STAG2 expression occurs in 15% of tumors and is associated with metastatic disease, suggesting a potential genetic vulnerability in Ewing Sarcoma.
Promiscuous partnerships in Ewing's sarcoma.
TLDR
This review aims to summarize the growing list of fusion oncogenes that characterize Ewing's sarcoma and Ewing’s-like tumors and highlights important questions that need to be answered to further support the existing concept that Ewings' sarcomA is strictly a "TET/ETS" fusion-driven malignancy.
Targeting the DNA repair pathway in Ewing sarcoma.
TLDR
Iinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide.
Therapeutic opportunities in Ewing sarcoma: EWS-FLI inhibition via LSD1 targeting
TLDR
This review examines targeting the chromatin regulatory enzymes recruited to conspire in oncogenesis with a focus on the histone lysine specific demethylase 1 (LSD1).
Genomic EWS-FLI1 Fusion Sequences in Ewing Sarcoma Resemble Breakpoint Characteristics of Immature Lymphoid Malignancies
TLDR
The occurrence of small deletions and duplications at the genomic junction is characteristic of involvement of the non-homologous end-joining (NHEJ) repair system, similar to findings at chromosomal breakpoints in pediatric leukemia and lymphoma.
Transcriptomic definition of molecular subgroups of small round cell sarcomas
TLDR
This study reports here the first unbiased and systematic search for gene fusions combined with unsupervised expression analysis of a series of 184 small round cell sarcomas, identifying biologically homogeneous groups of tumours and a completely novel group of epithelioid and spindle‐cell rhabdomyosarcomas characterized by EWSR1– or FUS–TFCP2 fusions.
Ewing Sarcoma Cells Secrete EWS/Fli-1 Fusion mRNA via Microvesicles
TLDR
Findings indicate the EWS/Fli-1 mRNA in MVs might be a new non-invasive diagnostic marker for specific cases of Ewing sarcoma.
Genomic landscape of Ewing sarcoma defines an aggressive subtype with co-association of STAG2 and TP53 mutations.
TLDR
Tumors that harbor STAG2 and TP53 mutations have a particularly dismal prognosis with current treatments and require alternative therapies, and novel drugs that target epigenetic regulators may constitute viable therapeutic strategies in a subset of patients with mutations in chromatin modifiers.
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