A Short note on Ewings sarcoma

Abstract

Ewing’s sarcoma was first distinguished as “diffuse endothelioma of bone”. The cause is still unknown the but mostly theories suggest that these tumors arise from a primitive cell obtained either from an embryologic tissue which is called the neural crest, or from mesenchymal stem cells that are capable to become one of a variety of tissue types. Pathologists have studied that Ewing sarcoma is almost alike to an even rarer soft tissue tumor called primitive neuroectodermal tumor (PNET).ES and PNET were having similar features when observed under microscope, in more than 95% of cases also had a similar genetic abnormality called as translocation. Hence they were grouped into class of cancers entitled Ewing’s Sarcoma Family of Tumor (ESFT).This family includes, Ewing’s sarcoma of the bone, Extraosseus Ewing’s sarcoma, Primitive neuroectodermal tumor (PNET),Peripheral neuroepithelioma, Askin’s tumor and Atypical Ewing’s sarcoma. The translocation in ESFT is between chromosomes 11 and 22 and is referred to as t (11;22). The gene from chromosome 22 encodes the Ewing sarcoma gene (EWS) whose function is not well-understood. The gene FLI1 from chromosome 11, is involved in turning other genes on and off. EWS/FLI is a fused gene, which encodes an altered fusion protein which controls the regulation of other genes that can give rise to cancers when inappropriately expressed.