Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Ewing Sarcoma

  • Heinrich Kovar
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_2042-2

Synonyms

Definition

Aggressive small round cell tumor affecting bone and soft tissue in children and young adults. Ewing’s sarcoma (ES) and peripheral primitive neuroectodermal tumor (pPNET), also called neuroepithelioma, are currently defined as biologically closely related tumors along a gradient of limited neuroglial differentiation. Askin tumor is the historical designation of Ewing’s sarcoma of the chest wall. Today, all these neoplasms are summarized as Ewing tumors (ET) or Ewing sarcoma family tumors (ESFT). Although first described in 1866 and 1890 by Lücke and Hildebrand, respectively, the disease carries the name of the American pathologist James Ewing who, in 1921, was the first to recognize the tumor as a separate entity, which he defined as diffuse endothelioma of the bone.

On the genetic level, Ewing sarcoma family tumors are defined by the consistent presence of a...

Keywords

Ewing Sarcoma Small Round Cell Ewing Sarcoma Cell Peripheral Primitive Neuroectodermal Tumor Ewing Sarcoma Family Tumor 
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. Bernstein M, Kovar H, Paulussen M et al (2006) Ewing’s sarcoma family of tumors: current management. Oncologist 11:503–519CrossRefPubMedGoogle Scholar
  2. Kovar H (2005) Context matters: the hen or egg problem in Ewing’s sarcoma. Semin Cancer Biol 15:189–9656CrossRefPubMedGoogle Scholar
  3. Kovar H (2014) Blocking the road, stopping the engine or killing the driver? Advances in targeting EWS/FLI-1 fusion in Ewing sarcoma as novel therapy. Expert Opin Ther Targets. 18:1315–28.CrossRefPubMedGoogle Scholar
  4. Lawlor ER and Sorensen PH (2015) Twenty Years on: What Do We Really Know about Ewing Sarcoma and What Is the Path Forward? Crit Rev Oncog 20:155–171.CrossRefPubMedPubMedCentralGoogle Scholar
  5. Gaspar N, Hawkins DS, Dirksen U et al. (2015) Ewing Sarcoma: Current Management and Future Approaches Through Collaboration. J Clin Oncol 33:3036–46.CrossRefPubMedGoogle Scholar
  6. Sand LG, Szuhai K, Hogendoorn PC (2015) Sequencing Overview of Ewing Sarcoma: A Journey across Genomic, Epigenomic and Transcriptomic Landscapes. Int J Mol Sci 16:16176–215.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Children’s Cancer Research InstituteViennaAustria