Central Nervous System Inflammatory Myofibroblastic Tumor

Preferred Label : Central Nervous System Inflammatory Myofibroblastic Tumor;

NCIt synonyms : Inflammatory Pseudotumor Intracranial;

NCIt definition : A multinodular intermediate fibroblastic neoplasm affecting the central nervous system. It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes and plasma cells.;

Neoplastic status : Undetermined;

Details

Origin ID

C7020;

UMLS CUI

C1334183;

Disease excludes normal cell origin
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
Disease may have findings
- Fascicular Pattern [NCIt concept]
- Fever [NCIt concept]
- Multinodular Lesion [NCIt concept]
- Myxoid Changes Present [NCIt concept]
- Pain [NCIt concept]
- Well-Circumscribed Lesion [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]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Cell [NCIt concept]
- Neoplastic Fibroblast [NCIt concept]
- Neoplastic Spindle-Shaped Fibroblast [NCIt concept]
- Neoplastic Spindle-Shaped Myofibroblast [NCIt concept]
disease_has_finding
- Eosinophilic Infiltrate [NCIt concept]
- Locally Aggressive Lesion [NCIt concept]
- Lymphocytic Infiltrate [NCIt concept]
- Plasmacytic Infiltrate [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
- ALK Gene [NCIt concept]
- CLTC/ALK Fusion Gene [NCIt concept]
- HMGA2 Gene [NCIt concept]
- TPM3/ALK Fusion Gene [NCIt concept]
- TPM4/ALK Fusion Gene [NCIt concept]
disease_may_have_abnormal_cell
- Ganglion-Like Cell [NCIt concept]
- Neoplastic Stellate Cell [NCIt concept]
disease_may_have_cytogenetic_abnormality
- t(1;2)(q25;p23) [NCIt concept]
- t(2;17)(p23;q23) [NCIt concept]
- t(2;19)(p23;p13) [NCIt concept]
disease_may_have_molecular_abnormality
- CLTC-ALK Fusion Protein Expression [NCIt concept]
- TPM3-ALK Fusion Protein Expression [NCIt concept]
- TPM4-ALK Fusion Protein Expression [NCIt concept]

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