Biphenotypic Sinonasal Sarcoma

Preferred Label : Biphenotypic Sinonasal Sarcoma;

NCIt synonyms : BSNS; Low-Grade Sinonasal Sarcoma with Neural and Myogenic Differentiation; Low Grade Sinonasal Sarcoma with Neural and Myogenic Differentiation; Sinonasal Biphenotypic Sarcoma;

NCIt definition : A rare, slow-growing, low-grade soft tissue sarcoma arising from the sinonasal tract. It presents with non-specific obstructive nasal symptoms. Morphologically, it is an infiltrative, cellular spindle cell neoplasm. It is associated with rearrangement of the PAX3 gene.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL977505;

Details

Origin ID

C164205;

UMLS CUI

C5230517;

Automatic exact mappings (from CISMeF team)
- Biphenotypic sinonasal sarcoma [ICD-11 Extension code]
- Biphenotypic sinonasal sarcoma (morphologic abnormality) [SNOMED CT concept]
Disease excludes normal cell origin
- Epithelial Cell [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]
- Sinonasal Tract [NCIt concept]
Has cytogenetic abnormalities
- PAX3 Gene Rearrangement [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Biphenotypic sinonasal sarcoma (morphologic abnormality) [SNOMED CT concept]
concept_is_in_subset
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Biphasic Pattern [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 Spindle Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Malignant Cellular Infiltrate [NCIt concept]
- Slow Growing Mass [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]
- Sinonasal Tract [NCIt concept]
disease_mapped_to_gene
- PAX3 Gene [NCIt concept]
- PAX3/MAML3 Fusion Gene [NCIt concept]
pathogenesis_of_disease_involves_gene
- PAX3 Gene [NCIt concept]
- PAX3 wt Allele [NCIt concept]
- PAX3/MAML3 Fusion Gene [NCIt concept]

Keyword's position in hierarchy(ies) :

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