Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

EWS-FLI (ets) Fusion Transcripts

  • Enrique de Alava
  • Santiago Ramón y Cajal
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_2046-3

Definition

The EWS-FLI1 fusion transcript is the result of a balanced reciprocal chromosomal translocation between chromosomes 11 and 22, which fuses the EWS gene in chromosome 22 to the FLI1 gene in chromosome 11. This fusion transcript is detected in approximately 85 % of cases of the Ewing sarcoma family of tumors and is considered a tumor-specific molecular rearrangement, therefore useful for diagnosis, prognosis, and presumably specific therapeutics. Approximately 10 % of Ewing tumors have fusions involving EWS and ERG genes. An additional 5 % bear fusions between EWS and other less frequent genes.

Characteristics

Structure

Chromosomal translocations result in the genesis of chimeric genes, encoding hybrid transcripts and novel fusion proteins. Many fusion proteins contain juxtaposed functional domains usually found in separate proteins. The EWS-FLI1 fusion protein contains the aminoterminal domain of EWS and the carboxyterminal region of FLI1. EWSgene is an RNA-binding protein,...

Keywords

Ewing Sarcoma Fusion Transcript Clear Cell Sarcoma Desmoplastic Small Round Cell Tumor Alveolar Rhabdomyosarcoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Alava E, de Gerald WL (2000) Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 18:204–213PubMedGoogle Scholar
  2. Hu-Lieskovan S, Heidel JD, Bartlett DW et al (2005) Sequence-specific knockdown of EWS-FLI1 by targeted, nonviral delivery of small interfering RNA inhibits tumor growth in a murine model of metastatic Ewing’s sarcoma. Cancer Res 65:8984–8992CrossRefPubMedGoogle Scholar
  3. Kovar H (2005) Context matters: the hen or egg problem in Ewing’s sarcoma. Semin Cancer Biol 15:189–196CrossRefPubMedGoogle Scholar
  4. Riggi N, Suva ML, Stamenkovic I (2006) Ewing’s Sarcoma-like tumors originate from EWS-FLI-1-expressing mesenchymal progenitor cells. Cancer Res 66:9786CrossRefGoogle Scholar
  5. Schleiermacher G, Peter M, Oberlin O et al (2003) Increased risk of systemic relapses associated with bone marrow micrometastasis and circulating tumor cells in localized Ewing tumor. J Clin Oncol 21:85–91CrossRefPubMedGoogle Scholar

See Also

  1. (2012) CD99. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 704. doi:10.1007/978-3-642-16483-5_941Google Scholar
  2. (2012) Chromosomal translocation t(9;22). In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, pp 845–846. doi:10.1007/978-3-642-16483-5_1142Google Scholar
  3. (2012) Formalin-fixed Paraffin-embedded Tissue. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1446. doi:10.1007/978-3-642-16483-5_2249Google Scholar
  4. (2012) Illegitimate recombination. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1808. doi:10.1007/978-3-642-16483-5_2960Google Scholar
  5. (2012) RT-PCR. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3322. doi:10.1007/978-3-642-16483-5_5129Google Scholar
  6. (2012) Transcript. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3752. doi:10.1007/978-3-642-16483-5_5898Google Scholar
  7. (2012) Transcription factor. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3752. doi:10.1007/978-3-642-16483-5_5901Google Scholar
  8. (2012) Translocation. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3773. doi:10.1007/978-3-642-16483-5_5942Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital /CSIC/University de SevillaSevilleSpain
  2. 2.Department of PathologyVall d’Hebron University HospitalBarcelonaSpain