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...
This is a preview of subscription content, log in via an institution to check access.
References
Bernstein M, Kovar H, Paulussen M et al (2006) Ewing’s sarcoma family of tumors: current management. Oncologist 11:503–519
Kovar H (2005) Context matters: the hen or egg problem in Ewing’s sarcoma. Semin Cancer Biol 15:189–9656
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.
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.
Gaspar N, Hawkins DS, Dirksen U et al. (2015) Ewing Sarcoma: Current Management and Future Approaches Through Collaboration. J Clin Oncol 33:3036–46.
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.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag Berlin Heidelberg
About this entry
Cite this entry
Kovar, H. (2015). Ewing Sarcoma. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_2042-2
Download citation
DOI: https://doi.org/10.1007/978-3-642-27841-9_2042-2
Received:
Accepted:
Published:
Publisher Name: Springer, Berlin, Heidelberg
Online ISBN: 978-3-642-27841-9
eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences