The DOI matches its Crossref record; 251 of the 251 references it cites were found in a registry or an index.
Ewing sarcoma
Abstract
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 are composed of small round cells expressing high levels of CD99. Genetically, they are characterized by balanced chromosomal translocations in which a member of the FET gene family is fused with an ETS transcription factor, with the most common fusion being EWSR1-FLI1 (85% of cases). Ewing sarcoma breakpoint region 1 protein (EWSR1)-Friend leukaemia integration 1 transcription factor (FLI1) is a tumour-specific chimeric transcription factor (EWSR1-FLI1) with neomorphic effects that massively rewires the transcriptome. Additionally, EWSR1-FLI1 reprogrammes the epigenome by inducing de novo enhancers at GGAA microsatellites and by altering the state of gene regulatory elements, creating a unique epigenetic signature. Additional mutations at diagnosis are rare and mainly involve STAG2, TP53 and CDKN2A deletions. Emerging studies on the molecular mechanisms of Ewing sarcoma hold promise for improvements in early detection, disease monitoring, lower treatment-related toxicity, overall survival and quality of life.
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“Outcomes remain poor for children and young adults with advanced solid tumors.1–5”
From Clinical Liquid Biopsy Testing for Detection of Actionable Genomic Alterations in Children and Young Adults With Advanced Solid Tumors on the GAIN Study (2026) · cites this paper as [3]
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Self-citation
Metadata
- Authors
- Grünewald, T. G. P., Cidre-Aranaz, F., Surdez, D., Tomazou, E. M., de Álava, E., Kovar, H., Sorensen, P. H., Delattre, O., & Dirksen, U.
- Affiliations
- German Cancer Research Center (DE)
- Inserm (FR)
- St. Anna Children's Cancer Research Institute (AT)
- University of British Columbia (CA)
- Corresponding author
- Grünewald, Thomas G. P.
- ORCID
- Venue
- Nature Reviews Disease Primers
- Published
- 2018-07-05
- Volume
- 4
- Issue
- 1
- Pages
- 5-5
- ISSN
- 2056-676X (ISSN-L)
- OpenAlex
- W4211227438
- Cited by
- 873
- References
- 250
- Citations by year
- 2026: 114 · 2025: 155 · 2024: 104 · 2023: 103 · 2022: 158 · 2021: 115 · 2020: 66 · 2019: 49 · 2018: 8
- FWCI
- 10.61
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- Locations
- Nature Reviews Disease Primers (publishedVersion)
- PubMed (publishedVersion)
- DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion)
- Topics
- Health Sciences › Medicine › Oncology › Sarcoma Diagnosis and Treatment
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Chromatin Remodeling and Cancer
- Health Sciences › Medicine › Hematology › Acute Lymphoblastic Leukemia research
- Keywords
- Ewing sarcoma, EWSR1-FLI1 fusion, CD99, epigenetic reprogramming, STAG2 mutation, TP53 mutation, CDKN2A deletion, treatment toxicity, cancer survivorship, soft tissue sarcoma
- MeSH terms
- 12E7 Antigen, Humans, Neoplasm Metastasis, Quality of Life, Radiography, Risk Factors, Sarcoma, Ewing, RNA-Binding Protein EWS, Proto-Oncogene Protein c-fli-1
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