Primary Intracranial Sarcoma, DICER1-Mutant

Preferred Label : Primary Intracranial Sarcoma, DICER1-Mutant;

NCIt definition : A primary intracranial sarcoma composed of malignant pleomorphic or spindle neoplastic cells typically demonstrating myogenic and/or chondroid differentiation. Cytoplasmic eosinophilic globules and myxoid stroma formation are usually present. It is associated with mutations in the DICER1 gene.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL1778762;

Details

Origin ID

C186610;

UMLS CUI

C5670660;

Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Cytoplasmic Eosinophilic Globules Present [NCIt concept]
- Myxoid Stroma Formation [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
- Lung [NCIt concept]
- Spinal Cord [NCIt concept]
Has associated anatomic sites
- Brain [NCIt concept]
- Central Nervous System [NCIt concept]
- Connective and Soft Tissue [NCIt concept]
- Nervous System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Malignant Cellular Infiltrate [NCIt concept]
disease_has_molecular_abnormality
- DICER1 Gene Mutation [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Brain [NCIt concept]
- Central Nervous System [NCIt concept]
disease_mapped_to_gene
- DICER1 Gene [NCIt concept]
disease_may_have_abnormal_cell
- Malignant Mesenchymal Spindle Cell [NCIt concept]
- Pleomorphic Connective and Soft Tissue Cell [NCIt concept]
pathogenesis_of_disease_involves_gene
- DICER1 Gene [NCIt concept]
- DICER1 wt Allele [NCIt concept]

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