Low Grade Fibromyxoid Sarcoma

Preferred Label : Low Grade Fibromyxoid Sarcoma;

NCIt related terms : Low-grade fibromyxoid sarcoma;

NCIt definition : A low-grade, late-metastasizing variant of fibrosarcoma characterized by alternating fibrous and myxoid areas and a whorling growth pattern. The neoplastic cells have a spindle morphology, and lack hyperchromasia or significant nuclear atypia. Approximately 40% of cases show the focal presence of collagen rosettes. A t(7;16)(q33;p11) translocation has been identified in the majority of cases, associated with the presence of FUS-CREB3L2 fusion protein. Rare cases carry the t(11;16)(p11;p11) translocation which is associated with the presence of the FUS-CREB3L1 fusion protein.;

Neoplastic status : Malignant;

Details

Origin ID

C45202;

UMLS CUI

C1275282;

Automatic exact mappings (from CISMeF team)
- Low grade fibromyxoid sarcoma [ICD-11 Extension code]
- Low grade fibromyxoid sarcoma (morphologic abnormality) [SNOMED CT concept]
Currated CISMeF NLP mapping
- Low-grade fibromyxoid sarcoma (disorder) [SNOMED CT concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
Disease may have findings
- Favorable Clinical Outcome [NCIt concept]
- Mild Cytologic Atypia [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
Manual NTBT mappings (CISMeF)
- fibromyxoid sarcoma [Disease (Nov.)]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Low grade fibromyxoid sarcoma (morphologic abnormality) [SNOMED CT concept]
- Low-grade fibromyxoid sarcoma (disorder) [SNOMED CT concept]
associated_with_malfunction_of_gene_product
- Cyclic AMP-Responsive Element-Binding Protein 3-Like Protein 1 [NCIt concept]
- Cyclic AMP-Responsive Element-Binding Protein 3-Like Protein 2 [NCIt concept]
concept_is_in_subset
- Cellosaurus Disease Terminology [NCIt concept]
- Cellosaurus Terminology [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
- Nuclear Pleomorphism [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Fibroblast [NCIt concept]
- Malignant Spindle-Shaped Fibroblast [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Cell [NCIt concept]
- Neoplastic Fibroblast [NCIt concept]
disease_has_finding
- Fibrotic Stroma Formation [NCIt concept]
- Low Grade Lesion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Myxoid Stroma Formation [NCIt concept]
- Whorling Growth Pattern [NCIt concept]
disease_has_normal_cell_origin
- Connective and Soft Tissue Cell [NCIt concept]
- Fibroblast [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
disease_mapped_to_gene
- FUS Gene [NCIt concept]
- FUS/CREB3L1 Fusion Gene [NCIt concept]
- FUS/CREB3L2 Fusion Gene [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Balanced Translocation [NCIt concept]
- t(11;16)(p11;p11) [NCIt concept]
- t(7;16)(q33;p11) [NCIt concept]
disease_may_have_molecular_abnormality
- FUS-CREB3L1 Fusion Protein Expression [NCIt concept]
- FUS-CREB3L2 Fusion Protein Expression [NCIt concept]
pathogenesis_of_disease_involves_gene
- FUS Gene [NCIt concept]
- FUS wt Allele [NCIt concept]
- FUS/CREB3L1 Fusion Gene [NCIt concept]
- FUS/CREB3L2 Fusion Gene [NCIt concept]

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