Terminology Service for NFDI4Health
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
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http://purl.obolibrary.org/obo/NCIT_C27291
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. [ ]
Term info
Label
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Synonyms
- EFTs
- Ewing Family of Tumors
- Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
- Ewing sarcoma family of tumors
- 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
- Tumors of Ewing's Family
- Tumors of the Ewing's Family
Subsets
NCIT_C126659, NCIT_C118168, NCIT_C102905, NCIT_C116977, NCIT_C103038
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.
Contributing Source
CTRP, CTEP
Display Name
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Legacy Concept Name
Ewing_s_Sarcoma_Peripheral_Primitive_Neuroectodermal_Tumor
Neoplastic Status
Malignant
Preferred Name
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Semantic Type
Neoplastic Process
UMLS CUI
C0684337
code
C27291
Term relations
Equivalent to:
- Embryonal Neoplasm and Disease_May_Have_Cytogenetic_Abnormality some t(11;22)(q24;q12) and Disease_May_Have_Molecular_Abnormality some EWSR1-FLI1 Fusion Protein Expression or Disease_May_Have_Cytogenetic_Abnormality some t(21;22)(q22;q12) and Disease_May_Have_Molecular_Abnormality some EWSR1-ERG Fusion Protein Expression or Disease_May_Have_Cytogenetic_Abnormality some t(7;22)(p22;q12) and Disease_May_Have_Molecular_Abnormality some EWSR1-ETV1 Fusion Protein Expression or Disease_May_Have_Cytogenetic_Abnormality some t(17;22)(q21;q12) and Disease_May_Have_Molecular_Abnormality some EWSR1-ETV4 Fusion Protein Expression or Disease_May_Have_Cytogenetic_Abnormality some t(16;21)(p11;q22) and Disease_May_Have_Molecular_Abnormality some FUS-ERG Fusion Protein Expression or Disease_May_Have_Cytogenetic_Abnormality some t(2;22)(q33;q12) and Disease_May_Have_Molecular_Abnormality some EWSR1-FEV Fusion Protein Expression or Disease_May_Have_Cytogenetic_Abnormality some t(1;22)(p36.1;q12) and Disease_May_Have_Cytogenetic_Abnormality some inv(22q12) and Disease_May_Have_Molecular_Abnormality some EWSR1-PATZ1 Fusion Protein Expression and Disease_Has_Normal_Tissue_Origin some Neural Crest and Disease_Has_Abnormal_Cell some Primitive Round to Oval Cell and Disease_May_Have_Abnormal_Cell some Malignant Small Round Cell and Disease_May_Have_Finding some Fever and Disease_May_Have_Finding some Hemorrhagic Change and Disease_May_Have_Finding some Homer Wright Rosette Formation and Disease_May_Have_Finding some Necrotic Change and Disease_May_Have_Associated_Disease some Anemia and Disease_Mapped_To_Gene some EWSR1 Gene and Disease_Mapped_To_Gene some EWSR1/FLI1 Fusion Gene and Disease_Mapped_To_Gene some EWSR1/ERG Fusion Gene and Disease_Mapped_To_Gene some EWSR1/FEV Fusion Gene and Disease_Mapped_To_Gene some EWSR1/PATZ1 Fusion Gene and Disease_Mapped_To_Gene some EWSR1/ETV1 Fusion Gene and Disease_Mapped_To_Gene some EWSR1/ETV4 Fusion Gene and Disease_Mapped_To_Gene some FUS/ERG Fusion Gene