Supratentorial Ependymoma, ZFTA-RELA Fusion-Positive

Preferred Label : Supratentorial Ependymoma, ZFTA-RELA Fusion-Positive;

NCIt synonyms : Ependymoma, ZFTA-RELA Fusion-Positive; Supratentorial Ependymoma, RELA Fusion-Positive; Supratentorial Ependymoma, ZFTA::RELA Fusion-Positive;

NCIt related terms : Ependymoma, RELA fusion-positive;

NCIt definition : A supratentorial ependymoma characterized by a gene fusion involving ZFTA and RELA genes. It accounts for the majority of supratentorial ependymomas in children. It has an unfavorable outcome when compared to other ependymoma subtypes.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL512399;

Détails

Origin ID

C129351;

UMLS CUI

C4289581;

Disease may have findings
- Calcification [NCIt concept]
- Unfavorable Clinical Outcome [NCIt concept]
Excludes anatomical site(s)
- Spinal Cord [NCIt concept]
Has associated anatomic sites
- Brain [NCIt concept]
- Central Nervous System [NCIt concept]
- Nervous System [NCIt concept]
- Supratentorial Brain [NCIt concept]
See also inter- (CISMeF)
- Ependymoma, RELA fusion-positive [ICD-11 Extension code]
- RELA fusion-positive ependymoma [ORDO Disease]
- RELA fusion-positive ependymoma (morphologic abnormality) [SNOMED CT concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- RELA fusion-positive ependymoma (morphologic abnormality) [SNOMED CT concept]
concept_is_in_subset
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
disease_excludes_finding
- Anaplastic Lesion [NCIt concept]
- Benign Cellular Infiltrate [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- No Evidence of YAP1 and ZFTA Gene Rearrangements Present [NCIt concept]
- Precise Histogenesis Unknown [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Ependymal Cell [NCIt concept]
- Neoplastic Glial Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell and Neoplastic Perineural Cell [NCIt concept]
disease_has_finding
- Low Grade Lesion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Monomorphic Cellular Infiltrate [NCIt concept]
- Slow Growing Mass [NCIt concept]
- Well-Circumscribed Lesion [NCIt concept]
disease_has_grade
- Grade 2 [NCIt concept]
disease_has_molecular_abnormality
- ZFTA Gene Rearrangement [NCIt concept]
- ZFTA-RELA Fusion Protein Expression [NCIt concept]
disease_has_normal_cell_origin
- Ependymal Cell [NCIt concept]
- Glial Cell [NCIt concept]
- Neuroepithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
disease_has_normal_tissue_origin
- Ependyma and Remnants of the Central Canal of the Spinal Cord [NCIt concept]
- Nerve Tissue, Neuroepithelial Tissue, and Nerve Sheaths [NCIt concept]
- Neuroepithelial Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Brain [NCIt concept]
- Central Nervous System [NCIt concept]
- Supratentorial Brain [NCIt concept]
disease_mapped_to_gene
- ZFTA Gene [NCIt concept]
- ZFTA/RELA Fusion Gene [NCIt concept]
pathogenesis_of_disease_involves_gene
- ZFTA/RELA Fusion Gene [NCIt concept]

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