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;

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.;

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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