Central Nervous System Solitary Fibrous Tumor

Preferred Label : Central Nervous System Solitary Fibrous Tumor;

NCIt synonyms : CNS Solitary Fibrous Tumor/Hemangiopericytoma; Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma; CNS Solitary Fibrous Tumor;

NCIt definition : A mesenchymal, non-meningothelial neoplasm arising from the central nervous system. It is characterized by a collagenous and low cellularity spindle cell and/or hemangiopericytomatous histopathological pattern, recurrent intrachromosomal rearrangement on chromosome 12q that results in the fusion of the NAB2 and STAT6 genes, high recurrence rates, and long-term risk of systemic metastasis.;

Neoplastic status : Undetermined;

NCI Metathesaurus CUI : CL512688;

Details

Origin ID

C129526;

UMLS CUI

C4329640;

Disease excludes normal cell origin
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
- Lung [NCIt concept]
Has associated anatomic sites
- Central Nervous System [NCIt concept]
- Connective and Soft Tissue [NCIt concept]
- Nervous System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
concept_is_in_subset
- NCIt Neoplasm Core Terminology [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Cell [NCIt concept]
- Neoplastic Fibroblast [NCIt concept]
- Neoplastic Spindle-Shaped to Round Cell [NCIt concept]
disease_has_finding
- Hyalinized Stroma Formation [NCIt concept]
- Nuclear Expression of STAT6 Protein [NCIt concept]
- Thin Bands of Collagen Present [NCIt concept]
- Thin-Walled Branching Vascular Pattern [NCIt concept]
disease_has_normal_cell_origin
- Connective and Soft Tissue Cell [NCIt concept]
- Fibroblast [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Central Nervous System [NCIt concept]
disease_mapped_to_gene
- NAB2/STAT6 Fusion Gene [NCIt concept]

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