Gastrointestinal Clear Cell Sarcoma/Malignant Gastrointestinal Neuroectodermal Tumor

Preferred Label : Gastrointestinal Clear Cell Sarcoma/Malignant Gastrointestinal Neuroectodermal Tumor;

NCIt synonyms : CCS/ GNET;

NCIt definition : A gastrointestinal tract sarcoma with neuroectodermal differentiation. It is characterized by the presence of neoplastic round cells with pale eosinophilic cytoplasm. Cytoplasmic clearing is seen only in a minority of cases. Half of the cases contain osteoclastic giant cells. It is associated with gene fusion translocations involving the EWSR1 gene, usually EWSR1-ATF1 fusion or EWSR1-CREB1 fusion.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL1406771;

Details

Origin ID

C172980;

UMLS CUI

C5417751;

Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Digestive 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]
- Neoplastic Round Cell [NCIt concept]
disease_has_finding
- Malignant Cellular Infiltrate [NCIt concept]
- Neuroendocrine Differentiation [NCIt concept]
disease_has_molecular_abnormality
- EWSR1 Gene Rearrangement [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Digestive System [NCIt concept]
- Gastrointestinal Tract [NCIt concept]
disease_mapped_to_gene
- EWSR1 Gene [NCIt concept]
- EWSR1/ATF1 Fusion Gene [NCIt concept]
- EWSR1/CREB1 Fusion Gene [NCIt concept]
disease_may_have_abnormal_cell
- Large Osteoclastic Giant Cell [NCIt concept]
disease_may_have_molecular_abnormality
- EWSR1-ATF1 Fusion Protein Expression [NCIt concept]
- EWSR1-CREB1 Fusion Protein Expression [NCIt concept]
pathogenesis_of_disease_involves_gene
- EWSR1 Gene [NCIt concept]
- EWSR1 wt Allele [NCIt concept]
- EWSR1/ATF1 Fusion Gene [NCIt concept]
- EWSR1/ATF1 Fusion Gene Type 1 [NCIt concept]
- EWSR1/ATF1 Fusion Gene Type 2 [NCIt concept]
- EWSR1/CREB1 Fusion Gene [NCIt concept]

Keyword's position in hierarchy(ies) :

You can consult :

recommendations
documents concerning education
documents for patients