Posterior Fossa Ependymoma, Group A (PFA)

Preferred Label : Posterior Fossa Ependymoma, Group A (PFA);

NCIt synonyms : Posterior Fossa Group A (PFA) Ependymoma, Pediatric-Type; Posterior Fossa Group A (PFA) Ependymoma; Group A Posterior Fossa Ependymoma, Pediatric-Type; Group A Posterior Fossa Ependymoma; Posterior Fossa Group A Ependymoma;

NCIt definition : A circumscribed ependymoma that arises in the posterior fossa with characteristic DNA methylation patterns, including CpG island hypermethylation, global DNA hypomethylation, reduction of nuclear H3 p.K28me3 (K27me3) expression, and EZHIP overexpression. It usually occurs in children younger than five years. PFA ependymomas have a worst prognosis compared to PFB ependymomas.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL1778957;

Details

Origin ID

C186450;

UMLS CUI

C5670548;

Disease may have findings
- Calcification [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]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_excludes_finding
- Anaplastic Lesion [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Precise Histogenesis Unknown [NCIt concept]
disease_has_abnormal_cell
- 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
- CpG Island Hypermethylation [NCIt concept]
- EZHIP Overexpression Positive [NCIt concept]
- Global DNA Hypomethylation [NCIt concept]
- Loss or Reduction of Nuclear Histone H3 K28me3 Expression [NCIt concept]
- Monomorphic Cellular Infiltrate [NCIt concept]
- Slow Growing Mass [NCIt concept]
- Well-Circumscribed Lesion [NCIt concept]
disease_has_grade
- Grade 2 [NCIt concept]
- Low Grade [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]
- Posterior Cranial Fossa [NCIt concept]

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