extraskeletal Ewing sarcoma/peripheral primitive neuroectodermal tumor

MONDO:0021039

Also Known As

extraosseous Ewing sarcoma/peripheral primitive neuroectodermal tumorextraosseous Ewing sarcoma/peripheral primitive neuroectodermal tumourextraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumorextraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumourextraosseous Ewings sarcoma-primitive neuroepithelial tumorextraosseous Ewings sarcoma-primitive neuroepithelial tumourextraskeletal Ewing sarcoma/peripheral primitive neuroectodermal tumorextraosseous Ewing's tumorextraosseous Ewing's tumour

Definition

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.