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Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Definition 1
A spectrum of malignant tumors, affecting mostly males under age 20, characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. These tumors may occur in the soft tissues or the bones. Pain and the presence of a mass are the most common clinical symptoms. (NCI Thesaurus)
Definition 2
A group of cancers that includes Ewing tumor of bone (ETB or Ewing sarcoma of bone), extraosseous Ewing (EOE) tumors, primitive neuroectodermal tumors (PNET or peripheral neuroepithelioma), and Askin tumors (PNET of the chest wall). These tumors all come from the same type of stem cell. (NCI Dictionary)
Definition 3
Round cell sarcomas with varying degrees of neuroectodermal differentiation. There is a recurrent cytogenetic aberration, most commonly t(11;22)(q24;q12) and t(21;22)(q22;q12). (NCI Thesaurus)
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