Ewing sarcoma/peripheral primitive neuroectodermal tumor
Ewing sarcoma/peripheral primitive neuroectodermal tumor
Definition
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.
Also known as EFTs, Ewing family of tumors, Ewing family of tumours, Ewing sarcoma family of tumors, Ewing sarcoma family of tumours, Ewing sarcoma/peripheral PNET, Ewing sarcoma/peripheral primitive neuroectodermal tumor, Ewing's family of tumors, Ewing's family of tumours, Ewing's sarcoma/peripheral primitive neuroectodermal tumor, Ewing's sarcoma/peripheral primitive neuroectodermal tumour, tumors of Ewing's family, tumors of the Ewing's family, tumours of Ewing's family, tumours of the Ewing's family — per MONDO