Ewing sarcoma family of tumors
Updates to Article Attributes
Ewing sarcoma family of tumours (ESFT) are a group of small round blue cell tumours that are closely histogenetically related, all demonstrating non-random t(11;22)(q24;q12) chromosome rearrangement resulting in the formation of the EWS-ETS fusion gene 1-3. They include:
- Ewing sarcoma of bone
- extraosseous Ewing sarcoma (EES)
- peripheral primitive neuroectodermal tumour (pPNET)
- Askin tumour
- neuroepithelioma
Although the literature is littered with contradictory terminology, all three tumours are likely to represent the same entity with minor differences in differentiation. For example, PNET are more differentiated demonstrating neural elements (e.g. Homer Wright rosettes) 2.
As such all three entities should be referred to as Ewing sarcoma, with pPNET / Askin representing extraskeletal Ewing sarcoma.
-<li><a href="/articles/ewing-sarcoma">Ewing sarcoma</a></li>- +<li><a href="/articles/ewing-sarcoma">Ewing sarcoma of bone</a></li>
- +<li>
- +<a title="Extraosseous Ewing sarcoma (EES)" href="/articles/extraskeletal-ewing-sarcoma">extraosseous Ewing sarcoma</a> (EES)</li>
References changed:
- 4. Zagar TM, Triche TJ, Kinsella TJ. Extraosseous Ewing's sarcoma: 25 years later. J. Clin. Oncol. 2008;26 (26): 4230-2. <a href="http://dx.doi.org/10.1200/JCO.2008.16.5308">doi:10.1200/JCO.2008.16.5308</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18779607">Pubmed citation</a><span class="auto"></span>