/Ewing sarcoma/peripheral primitive neuroectodermal tumor

Ewing sarcoma/peripheral primitive neuroectodermal tumor

MONDO:0021038

Also Known As

EFTsEwing family of tumorsEwing family of tumoursEwing sarcoma family of tumorsEwing sarcoma family of tumoursEwing sarcoma/peripheral PNETEwing sarcoma/peripheral primitive neuroectodermal tumorEwing's family of tumorsEwing's family of tumoursEwing's sarcoma/peripheral primitive neuroectodermal tumorEwing's sarcoma/peripheral primitive neuroectodermal tumourtumors of Ewing's familytumors of the Ewing's familytumours of Ewing's familytumours of the Ewing's family

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.