NTRK-Rearranged Spindle Cell Neoplasm

Preferred Label : NTRK-Rearranged Spindle Cell Neoplasm;

NCIt synonyms : NTRK-Positive Tumor Resembling Peripheral Nerve Sheath Tumor; Lipofibromatosis-Like Neural Tumor; NTRK-Rearranged Mesenchymal Tumor; SCN-NTRK; Spindle Cell Neoplasm, NTRK-Rearranged;

NCIt definition : An emerging rare group of soft tissue tumors, usually characterized by a monomorphic spindle cell infiltrate, stromal hyalinization, and frequent coexpression of S100 and CD34 by immunohistochemistry. Most tumors harbor NTRK1 fusions with a variety of partners. Rare cases involving NTRK2 and NTRK3 fusions have also been reported. The tumors usually arise from the extremities, trunk, and head and neck area, and less often within body cavities.;

Neoplastic status : Undetermined;

Concept status : Provisional_Concept;

NCI Metathesaurus CUI : CL1648150;

Details

Origin ID

C178426;

UMLS CUI

C5555025;

Currated CISMeF NLP mapping
- lipofibromatosis-like neural tumor [DO Concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
Has cytogenetic abnormalities
- NTRK Gene Rearrangement [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Spindle Cell [NCIt concept]
disease_has_finding
- CD34-Positive Neoplastic Cells Present [NCIt concept]
- Hyalinized Stroma Formation [NCIt concept]
- Monomorphic Cellular Infiltrate [NCIt concept]
- S-100-Positive Neoplastic Cells Present [NCIt concept]
- Tissue Differentiation Uncertain [NCIt concept]
disease_has_normal_tissue_origin
- Soft Tissue [NCIt concept]

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