Poorly Differentiated Chordoma

Preferred Label : Poorly Differentiated Chordoma;

NCIt definition : A rare, aggressive type of chordoma characterized by loss of SMARCB1 expression. It affects children and occasionally young adults. Females are affected twice as frequently as males. It usually arises in the axial skeleton. It is composed of sheets or nests of malignant epithelioid cells with abundant eosinophilic cytoplasm. The prognosis is poor.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL1647483;

Details

Origin ID

C177898;

UMLS CUI

C5554730;

Disease excludes normal cell origin
- Bone Marrow Stem Cell [NCIt concept]
Has associated anatomic sites
- Bone [NCIt concept]
- Connective and Soft Tissue [NCIt concept]
- Musculoskeletal System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
disease_excludes_normal_tissue_origin
- Germinal Layer [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Epithelioid Cell with Abundant Eosinophilic Cytoplasm [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Aggressive Clinical Course [NCIt concept]
- Geographic Necrosis [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Loss of INI 1 Protein Expression [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Nuclear Pleomorphism [NCIt concept]
disease_has_molecular_abnormality
- SMARCB1 Gene Deletion [NCIt concept]
disease_has_normal_cell_origin
- Embryonic Cell [NCIt concept]
disease_has_normal_tissue_origin
- Embryonic Tissue [NCIt concept]
- Remnants of the Notochord [NCIt concept]
disease_has_primary_anatomic_site
- Bone [NCIt concept]
disease_mapped_to_gene
- SMARCB1 Gene [NCIt concept]
pathogenesis_of_disease_involves_gene
- SMARCB1 Gene [NCIt concept]
- SMARCB1 wt Allele [NCIt concept]

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