Orbital Extraskeletal Myxoid Chondrosarcoma

Preferred Label : Orbital Extraskeletal Myxoid Chondrosarcoma;

NCIt synonyms : Orbit NR4A3-Rearranged Myxoid Sarcoma; Orbit Extraskeletal Myxoid Chondrosarcoma; Orbital NR4A3-Rearranged Myxoid Sarcoma;

NCIt definition : An extraskeletal myxoid chondrosarcoma that arises from the orbit. The orbit is the most frequently affected site for extraskeletal myxoid chondrosarcomas in the head and neck region.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL1921716;

Details

Origin ID

C202882;

UMLS CUI

C5908835;

Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Cord-Like Pattern [NCIt concept]
- Cribriform Pattern [NCIt concept]
- Cystic Change [NCIt concept]
- Hemorrhagic Change [NCIt concept]
- Spindle Cell Pattern [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
- Maxillofacial Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Head and Neck [NCIt concept]
- Orbit [NCIt concept]
Has cytogenetic abnormalities
- NR4A3 Gene Rearrangement [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]
- Malignant Cell with Eosinophilic Cytoplasm [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Extraosseous Lesion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Metastatic/Recurrent Disease [NCIt concept]
- Multinodular Pattern [NCIt concept]
- Myxoid Stroma Formation [NCIt concept]
- Tissue Differentiation Uncertain [NCIt concept]
- Well-Circumscribed Lesion [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Head and Neck [NCIt concept]
- Orbit [NCIt concept]
disease_mapped_to_gene
- EWSR1/NR4A3 Fusion Gene [NCIt concept]
- NR4A3 Gene [NCIt concept]
- TAF15/NR4A3 Fusion Gene [NCIt concept]
- TCF12/NR4A3 Fusion Gene [NCIt concept]
- TFG/NR4A3 Fusion Gene [NCIt concept]
disease_may_have_cytogenetic_abnormality
- t(3;9)(q11-12;q22) [NCIt concept]
- t(9;15)(q22;q21) [NCIt concept]
- t(9;17)(q22;q11) [NCIt concept]
- t(9;22)(q22-31;q12) [NCIt concept]
disease_may_have_molecular_abnormality
- EWSR1-NR4A3 Fusion Protein Expression [NCIt concept]
- TAF15-NR4A3 Fusion Protein Expression [NCIt concept]
- TCF12-NR4A3 Fusion Protein Expression [NCIt concept]
- TFG-NR4A3 Fusion Protein Expression [NCIt concept]

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