NF1-Associated Malignant Peripheral Nerve Sheath Tumor

Preferred Label : NF1-Associated Malignant Peripheral Nerve Sheath Tumor;

NCIt definition : A malignant peripheral nerve sheath tumor that is associated with NF1 gene inactivation and a history of neurofibromatosis type 1.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL977440;

Details

Origin ID

C164313;

UMLS CUI

C5236143;

Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Aggressive Clinical Course [NCIt concept]
- Geographic Necrosis [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Nervous System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
- Radiation Exposure [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Hyperchromatic Spindle Cell [NCIt concept]
- Malignant Cell [NCIt concept]
- Malignant Perineural Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell and Neoplastic Perineural Cell [NCIt concept]
- Neoplastic Perineural Cell [NCIt concept]
disease_has_associated_disease
- Neurofibromatosis Type 1 [NCIt concept]
disease_has_finding
- Diffuse Pattern [NCIt concept]
- Firm Mass [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- NF1 Mutation Positive Malignant Peripheral Nerve Sheath Tumor [NCIt concept]
- Necrotic Change [NCIt concept]
- Pseudoencapsulated Mass [NCIt concept]
disease_has_molecular_abnormality
- NF1 Gene Inactivation [NCIt concept]
disease_has_normal_cell_origin
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
- Perineural Cell [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Nerve Tissue and Nerve Sheaths [NCIt concept]
- Nerve Tissue, Neuroepithelial Tissue, and Nerve Sheaths [NCIt concept]
disease_has_primary_anatomic_site
- Peripheral Nerve [NCIt concept]
disease_mapped_to_gene
- NF1 Gene [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Loss of Chromosome 22 [NCIt concept]
disease_may_have_molecular_abnormality
- TP53 Gene Inactivation [NCIt concept]
may_be_associated_disease_of_disease
- Neurofibromatosis Type 1 [NCIt concept]
- Neurofibromatosis Type 1 with Inoperable, Progressive, Symptomatic Plexiform Neurofibromas [NCIt concept]
- Recurrent Neurofibromatosis Type 1 [NCIt concept]
- Refractory Neurofibromatosis Type 1 [NCIt concept]
pathogenesis_of_disease_involves_gene
- NF1 Gene [NCIt concept]
- NF1 wt Allele [NCIt concept]

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