GLI1-Altered Soft Tissue Tumor

Preferred Label : GLI1-Altered Soft Tissue Tumor;

NCIt synonyms : Pericytoma with t(7;12); GAESTT; GLI1-Altered Epithelioid Soft Tissue Tumor; Epithelioid Neoplasm with GLI1 Rearrangement;

NCIt definition : A rare mesenchymal neoplasm of uncertain histogenesis that arises from soft tissue and viscera. It is characterized by the presence of nests and sheets of neoplastic epithelioid cells separated by a rich delicate arborizing vascular network, and GLI1 gene rearrangement. It presents as a slow-growing painless mass. The majority of cases have an indolent clinical course following complete excision. Distant metastases have been reported in a minority of cases.;

Neoplastic status : Undetermined;

Details

Origin ID

C202896;

Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
Has cytogenetic abnormalities
- GLI1 Gene Rearrangement [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Epithelioid Cell [NCIt concept]
disease_has_finding
- Delicate Arborizing Vascular Channel Formation [NCIt concept]
- Locally Aggressive Lesion [NCIt concept]
- Precise Histogenesis Unknown [NCIt concept]
disease_has_normal_cell_origin
- Cell [NCIt concept]
disease_has_normal_tissue_origin
- Soft Tissue [NCIt concept]
- Tissue [NCIt concept]
disease_mapped_to_gene
- ACTB/GLI1 Fusion Gene [NCIt concept]
- GLI1 Gene [NCIt concept]
pathogenesis_of_disease_involves_gene
- ACTB/GLI1 Fusion Gene [NCIt concept]
- GLI1 Gene [NCIt concept]
- GLI1 wt Allele [NCIt concept]
- tGLI1 Allele [NCIt concept]

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