Polymorphous Low Grade Neuroepithelial Tumor of the Young

Preferred Label : Polymorphous Low Grade Neuroepithelial Tumor of the Young;

NCIt synonyms : Polymorphous Low-Grade Neuroepithelial Tumor of the Young; PLNTY; Diffuse or Nonspecific Form of Dysembryoplastic Neuroepithelial Tumor; Long-Term Epilepsy-Associated Tumor; Massively Calcified Low-Grade Glioma; Massively Calcified Low Grade Glioma;

NCIt definition : A low-grade cerebral tumor associated with seizures and in many cases refractory epilepsy. It usually occurs in the second and third decades of life. It is characterized by the presence of oligodendroglioma-like components. It may also contain astrocytic components. MAPK pathway-activating genetic alterations play a role in the development of this tumor. Causative gene alterations include mutations resulting in the expression of BRAF p.V600E and gene fusions involving FGFR2 or FGFR3 genes. IDH gene mutations and 1p/19q codeletion are not present.;

Neoplastic status : Undetermined;

NCI Metathesaurus CUI : CL1662794;

Details

Origin ID

C180378;

UMLS CUI

C5556330;

Disease excludes normal cell origin
- Oligodendrocyte [NCIt concept]
Disease may have findings
- Seizure [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]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
disease_excludes_finding
- Precise Histogenesis Unknown [NCIt concept]
disease_excludes_molecular_abnormality
- 1p/19q Codeletion [NCIt concept]
- IDH Gene Family Mutation [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell and Neoplastic Perineural Cell [NCIt concept]
disease_has_finding
- CD34-Positive Neoplastic Cells Present [NCIt concept]
- Low Grade Lesion [NCIt concept]
- MAPK Pathway Activating Gene Alteration Positive [NCIt concept]
- Oligodendroglioma-Like Component with Perinuclear Cytoplasmic Halos Present [NCIt concept]
disease_has_grade
- Low Grade [NCIt concept]
disease_has_normal_cell_origin
- Neuroepithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
disease_has_normal_tissue_origin
- 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]
disease_may_have_molecular_abnormality
- BRAF Gene Mutation [NCIt concept]
- FGFR2 Gene Mutation [NCIt concept]
- FGFR3 Gene Mutation [NCIt concept]

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