Malignant Peripheral Nerve Sheath Tumor with Perineurial Differentiation

Preferred Label : Malignant Peripheral Nerve Sheath Tumor with Perineurial Differentiation;

NCIt synonyms : Malignant Perineurioma; Perineurial Malignant Peripheral Nerve Sheath Tumor;

NCIt related terms : Perineural MPNST; Perineurioma, malignant;

NCIt definition : A very rare malignant tumor with morphologic features similar to those of benign perineurioma of soft tissue along with hypercellularity, nuclear atypia, hyperchromasia, and a high mitotic rate.;

Neoplastic status : Malignant;

ICD-O code : 9571/3;

Details

Origin ID

C66845;

UMLS CUI

C1266188;

Automatic exact mappings (from CISMeF team)
- Malignant perineurioma [ICD-11 Category]
- Malignant peripheral nerve sheath neoplasm with perineurial differentiation (disorder) [SNOMED CT concept]
- Perineurioma, malignant [ICD-O code]
- Perineurioma, malignant [ICD-11 Extension code]
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]
UMLS correspondences (same concept)
- Malignant peripheral nerve sheath neoplasm with perineurial differentiation (disorder) [SNOMED CT concept]
- Malignant peripheral nerve sheath tumor with perineurial differentiation [ORDO Disease]
- Perineurioma, malignant (morphologic abnormality) [SNOMED CT concept]
concept_is_in_subset
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology Terminology [NCIt concept]
- Mapped ICDO3.1 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]
- 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
- 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]
- Neoplastic Perineurial Cell [NCIt concept]
disease_has_finding
- Diffuse Pattern [NCIt concept]
- Firm Mass [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Hyperchromasia [NCIt concept]
- Increased Cellularity Present [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Necrotic Change [NCIt concept]
- Nuclear Atypia [NCIt concept]
- Pseudoencapsulated Mass [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_may_have_cytogenetic_abnormality
- Loss of Chromosome 22 [NCIt concept]
disease_may_have_molecular_abnormality
- TP53 Gene Inactivation [NCIt concept]

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