About: Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

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Attributes	Values
rdf:type	Class
rdfs:label	Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
equivalentClass	nodeID://b632459
Concept_In_Subset	NCI CTEP Simplified Disease Classification Terminology NCI CTEP SDC Ewing Sarcoma Sub-Category Terminology
Semantic_Type	Neoplastic Process
Preferred_Name	Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
NCI_META_CUI	CL033142
Contributing_Source	CTEP
ALT_DEFINITION	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-GLOSS 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
Neoplastic_Status	Malignant
Legacy_Concept_Name	Ewing_s_Sarcoma_Peripheral_Primitive_Neuroectodermal_Tumor
FULL_SYN	Ewing Family of TumorsSYNCI EFTsPTNCI-GLOSSCDR0000570758 Ewing's Family of TumorsSYNCI Tumors of Ewing's FamilySYNCI Ewing's Family of TumoursSYNCI Ewing sarcoma/Peripheral PNETSYCTEP10015563 Ewing Sarcoma/Peripheral Primitive Neuroectodermal TumorPTNCI Tumors of the Ewing's FamilySYNCI Ewing sarcoma/Peripheral primitive neuroectodermal tumorPTCTEP10015563 Ewing's Sarcoma/Peripheral Primitive Neuroectodermal TumorSYNCI Ewing sarcoma family of tumorsPTNCI-GLOSSCDR0000322134
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.NCI
code	C27291
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