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Cited by 880Sarcoma Diagnosis and TreatmentChromatin Remodeling and CancerAcute Lymphoblastic Leukemia research

The DOI matches its Crossref record; 251 of the 251 references it cites were found in a registry or an index.

Ewing sarcoma

Grünewald, Thomas G. P., Cidre-Aranaz, Florencia, Surdez, Didier, Tomazou, Eleni M., de Álava, Enrique, Kovar, Heinrich, Sorensen, Poul H., Delattre, Olivier, Dirksen, Uta·2018·Nature Reviews Disease Primers
DOI 10.1038/s41572-018-0003-x
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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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Crossref · 10.1038/s41572-018-0003-x

Cites

251 of 251 references verified0 citation errors · 0 not checkable

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Citations

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Research works printed but not linked: 8

  1. Ewing, J. Diffuse endothelioma of bone. Proc. N. Y. Pathol. Soc. 21, 17 (1921).
  2. Lessnick, S. L., Braun, B. S., Denny, C. T. & May, W. A. Multiple domains mediate transformation by the Ewing’s sarcoma EWS/FLI-1 fusion gene. Oncogene 10, 423–431 (1995).
  3. Ohno, T., Rao, V. N. & Reddy, E. S. EWS/Fli-1 chimeric protein is a transcriptional activator. Cancer Res. 53, 5859–5863 (1993).
  4. Pizzo, P. A. & Poplack, D. G. (eds) Principles and Practice of Pediatric Oncology 7th edn (Lippincott Wililiams & Wilkins, Philadelphia, 2015).
  5. ESMO/European Sarcoma Network Working Group. Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann. Oncol. 25 (Suppl 3), iii113–iii123 (2014).
  6. Machado, I., López-Guerrero, J. A., Scotlandi, K., Picci, P. & Llombart-Bosch, A. Immunohistochemical analysis and prognostic significance of PD-L1, PD-1, and CD8+ tumor-infiltrating lymphocytes in Ewing’s sarcoma family of tumors (ESFT). Virchows Arch. Int. J. Pathol. 472, 815–824 (2018).
  7. Wang, Y. X. et al. Inhibiting platelet-derived growth factor beta reduces Ewing’s sarcoma growth and metastasis in a novel orthotopic human xenograft model. In Vivo 23, 903–909 (2009).
  8. Bhan, A. & Mandal, S. S. LncRNA HOTAIR: a master regulator of chromatin dynamics and cancer. Biochim. Biophys. Acta 1856, 151–164 (2015).

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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
Publisher
Springer Science and Business Media LLC
Published
2018-07-05
Volume
4
Issue
1
Pages
5-5
ISSN
2056-676X (ISSN-L)
DOI
10.1038/s41572-018-0003-x
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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