Health / Medical Topics

    Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

    Definition 1

    A spectrum of malignant tumors arising from the soft tissues, 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. Pain and the presence of a mass are the most common clinical symptoms. (NCI Thesaurus)

    Definition 2

    Round cell sarcomas of soft tissue 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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