About: Malignant Peripheral Nerve Sheath Tumor

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Attributes	Values
rdf:type	Class
rdfs:label	Malignant Peripheral Nerve Sheath Tumor
equivalentClass	nodeID://b635267
Concept_In_Subset	NCI CTEP Simplified Disease Classification Terminology NCI CTEP SDC Non-Rhabdomyosarcoma Soft Tissue Sarcoma Sub-Category Terminology Clinical Data Interchange Standards Consortium Terminology CDISC SDTM Terminology CDISC SEND Terminology CDISC SEND Tumor Findings Results Terminology
Semantic_Type	Neoplastic Process
Preferred_Name	Malignant Peripheral Nerve Sheath Tumor
UMLS_CUI	C0751690
Contributing_Source	CDISC CTEP
ALT_DEFINITION	Any malignant tumor arising from a peripheral nerve or showing nerve sheath differentiation, with the exception of tumors originating from the epineurium or the peripheral nerve vasculature. Approximately 50% of cases are associated with neurofibromatosis 1.NCI A malignant neoplasm, originating from the sheaths of the peripheral nerve.CDISC 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).NCI-GLOSS
ICD-O-3_Code	9540/3 9560/3
Neoplastic_Status	Malignant
Legacy_Concept_Name	Malignant_Peripheral_Nerve_Sheath_Tumor
FULL_SYN	Malignant Peripheral Nerve Sheath NeoplasmSYNCI MALIGNANT NEURILEMOMASYCDISC Malignant NeurilemmomaSYNCI Malignant Peripheral Nerve Sheath TumorSYNCITCGA MALIGNANT NEURILEMMOMASYCDISC MALIGNANT NEOPLASM OF PERIPHERAL NERVE SHEATHSYCDISC Malignant Neoplasm of Peripheral Nerve SheathSYNCI MPNSTPTNCI-GLOSSCDR0000335498 MALIGNANT TUMOR OF THE PERIPHERAL NERVE SHEATHSYCDISC Malignant Tumor of the Peripheral Nerve SheathSYNCI malignant peripheral nerve sheath tumorPTNCI-GLOSSCDR0000335496 MPNSTABNCI NeurofibrosarcomaSYNCI MALIGNANT NEOPLASM OF THE PERIPHERAL NERVE SHEATHSYCDISC NEUROFIBROSARCOMA, MALIGNANTSYCDISC Malignant Peripheral Nerve Sheath TumorPTNCI MPNSTSYCDISC Malig. periph. nerve sheath tum.SYCTEP10026667 Malignant Neoplasm of the Peripheral Nerve SheathSYNCI PERIPHERAL NERVE SHEATH TUMOR, MALIGNANTPTCDISC MALIGNANT PERIPHERAL NERVE SHEATH TUMOURSYCDISC Malignant Peripheral Nerve Sheath TumourSYNCI Malignant SchwannomaSYNCI Malignant peripheral nerve sheath tumorPTCTEP10026667 MALIGNANT SCHWANNOMASYCDISC Malignant NeurilemomaSYNCI MALIGNANT TUMOR OF PERIPHERAL NERVE SHEATHSYCDISC MALIGNANT PERIPHERAL NERVE SHEATH NEOPLASMSYCDISC Malignant Tumor of Peripheral Nerve SheathSYNCI
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).NCI
code	C3798
is rdf:first of	nodeID://b734235 nodeID://b734236 nodeID://b734237 nodeID://b734238 nodeID://b734239 nodeID://b734240 nodeID://b734241 nodeID://b734242 nodeID://b734243 nodeID://b734244 nodeID://b734245 nodeID://b734246 nodeID://b734247 nodeID://b734248
is rdfs:subClassOf of	Malignant Peripheral Nerve Sheath Tumor with Perineurial Differentiation
is someValuesFrom of	nodeID://b648892 nodeID://b695105 nodeID://b696488 nodeID://b698329

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