Malignant Peripheral Nerve Sheath Tumor

Preferred Label : Malignant Peripheral Nerve Sheath Tumor;

NCIt synonyms : MPNST; Malignant Schwannoma; Malignant Peripheral Nerve Sheath Neoplasm;

NCIt related terms : Neurilemoma, malignant; SCHWANNOMA, MALIGNANT; MPNST, NOS; Neurofibrosarcoma; Malignant Peripheral Nerve Sheath Tumour; Malignant Neurilemmoma; Malig. periph. nerve sheath tum.; Neurogenic sarcoma; Neurofibrosarcoma, Malignant;

NCIt definition : An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region. It may be associated with neurofibromatosis 1 (NF1).;

Alternative definition : NCI-GLOSS: A type of soft tissue sarcoma that develops in cells that form a protective sheath (covering) around peripheral nerves, which are nerves that are outside of the central nervous system (brain and spinal cord).; CDISC: A malignant neoplasm, originating from the sheaths of the peripheral nerve.;

Neoplastic status : Malignant;

ICD-O code : 9560/3; 9540/3;

Codes from synonyms : CDR0000335496; CDR0000335498; 10026667;

Details

Origin ID

C3798;

UMLS CUI

C0751690;

Automatic exact mappings (from CISMeF team)
- Malignant peripheral nerve sheath tumour [ICD-11 Extension code]
- Malignant peripheral nerve sheath tumour of cranial or paraspinal nerves [ICD-11 Sub-sub category]
- Neurilemmoma, malignant [ICD-9 code]
- Neurilemoma, malignant [ICD-11 Extension code]
- Neurofibrosarcoma [PASCAL descriptor]
- Neurofibrosarcoma [ICD-9 code]
- malignant peripheral nerve sheath tumor [Radlex concept]
- neurofibrosarcoma [DO Concept]
Currated CISMeF NLP mapping
- Malignant peripheral nerve sheath tumor [ICD-O code]
- Malignant peripheral nerve sheath tumor [ORDO Disease]
- Malignant peripheral nerve sheath tumor [MedDRA LLT]
- Malignant peripheral nerve sheath tumor (disorder) [SNOMED CT concept]
- Malignant peripheral nerve sheath tumor (morphologic abnormality) [SNOMED CT concept]
- Malignant peripheral nerve sheath tumour [MedDRA LLT]
- Neurilemmoma malignant [MedDRA LLT]
- Neurilemmoma, malignant [ICD-O code]
- Neurilemoma malignant [MedDRA LLT]
- Neurilemoma, malignant (morphologic abnormality) [Inactive SNOMED CT concept]
- Neurofibrosarcoma NOS [MedDRA LLT]
- Peripheral nerve sheath tumor malignant [MedDRA LLT]
- Peripheral nerve sheath tumour malignant [MedDRA LLT]
- Peripheral nerve sheath tumour malignant [MedDRA Preferred Term]
- Schwannoma malignant [MedDRA LLT]
- malignant peripheral nerve sheath tumor [DO Concept]
- malignant peripheral nerve sheath tumors [MeSH concept]
- neurilemmoma [MeSH Descriptor]
- neurilemmoma, malignant [SNOMED Notion]
- neurofibrosarcoma [SNOMED Notion]
- peripheral nerve sheath cancer [Disease (Nov.)]
DO Cross reference
- malignant peripheral nerve sheath tumor [DO Concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Aggressive Clinical Course [NCIt concept]
- Geographic Necrosis [NCIt concept]
HPO term
- Neurofibrosarcoma [HPO term]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Nervous System [NCIt concept]
See also inter- (CISMeF)
- Neurofibrosarcoma [HPO term]
- Neurofibrosarcoma [MedDRA Preferred Term]
- Neurofibrosarcomas [MedDRA High Level Term]
- neurofibrosarcoma [MeSH Descriptor]
- neurofibrosarcoma [MeSH concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Malignant peripheral nerve sheath tumor [MedDRA LLT]
- Malignant peripheral nerve sheath tumor [ICD-O code]
- Malignant peripheral nerve sheath tumor (disorder) [SNOMED CT concept]
- Malignant peripheral nerve sheath tumor (morphologic abnormality) [SNOMED CT concept]
- Malignant peripheral nerve sheath tumour [MedDRA LLT]
- Neurilemmoma malignant [MedDRA LLT]
- Neurilemoma malignant [MedDRA LLT]
- Neurilemoma, malignant (morphologic abnormality) [Inactive SNOMED CT concept]
- Peripheral nerve sheath tumor malignant [MedDRA LLT]
- Peripheral nerve sheath tumour malignant [MedDRA Preferred Term]
- Peripheral nerve sheath tumour malignant [MedDRA LLT]
- Schwannoma malignant [MedDRA LLT]
- malignant peripheral nerve sheath tumor [DO Concept]
- malignant peripheral nerve sheath tumors [MeSH concept]
- neurilemmoma, malignant [SNOMED Notion]
- peripheral nerve sheath cancer [Disease (Nov.)]
Validated automatic mappings to NTBT
- nerve sheath neoplasms [MeSH Descriptor]
associated_with_malfunction_of_gene_product
- Bone Morphogenetic Protein 3 [NCIt concept]
concept_is_in_subset
- CDISC SEND Terminology [NCIt concept]
- CDISC SEND Tumor Findings Results Terminology [NCIt concept]
- CPTAC Baseline Medical Forms Terminology [NCIt concept]
- CPTAC Codelists Terminology [NCIt concept]
- CPTAC Neoplasms Codelist [NCIt concept]
- CPTAC Sarcoma Baseline Form [NCIt concept]
- CPTAC Terminology [NCIt concept]
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
- Cellosaurus Disease Terminology [NCIt concept]
- Cellosaurus Terminology [NCIt concept]
- Clinical Data Interchange Standards Consortium Terminology [NCIt concept]
- 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]
- NCI CTEP SDC Non-Rhabdomyosarcoma Soft Tissue Sarcoma Sub-Category Terminology [NCIt concept]
- NCI CTEP Simplified Disease Classification Terminology [NCIt concept]
- NCIt Neoplasm Core 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]
disease_has_associated_gene
- BMP3 Gene [NCIt concept]
- BMP3 wt Allele [NCIt concept]
disease_has_finding
- Diffuse Pattern [NCIt concept]
- Firm Mass [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Necrotic Change [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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