Atlas One · Host Biology · Scale 07 — System

MPNST

MPNST is the most lethal NF1-associated tumor; NF1 LOF + CDKN2A deletion + PRC2/SUZ12 LOF → H3K27me3 loss defines high-grade MPNST; ~50% sporadic; surgery is the only curative modality; 5-year OS ~25-40%; selumetinib active in NF1 plexiform neurofibromas but not MPNST.

Also known as: MPNST, malignant peripheral nerve sheath tumor, NF1 MPNST, neurofibrosarcoma, malignant schwannoma, sarcoma NF1, plexiform neurofibroma malignant transformation, MPNST SUZ12, MPNST H3K27me3, MPNST PRC2

Scale 07 — SystemScale
draftStatus
2026-06-06Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
mpnst

MPNST

MPNST is the most lethal NF1-associated tumor; NF1 LOF + CDKN2A deletion + PRC2/SUZ12 LOF → H3K27me3 loss defines high-grade MPNST; ~50% sporadic; surgery is the only curative modality; 5-year OS ~25-40%; selumetinib active in NF1 plexiform neurofibromas but not MPNST.

Entry Metadata

FieldValue
IDmpnst
NameMPNST
Statusdraft
Last reviewed2026-06-06
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toNf1 connects-toCdkn2A connects-toEzh2 connects-toErk1 2 connects-toNeurofibromatosis Type 1 connects-toPeripheral Nerve connects-toAtypical Teratoid Rhabdoid Tumor connects-toSchwannomatosis connects-toSynovial Sarcoma connects-toMusculoskeletal System connects-toPhoton connects-toRhabdomyosarcoma connects-toEwing Sarcoma connects-toGist connects-toLi Fraumeni Syndrome connects-toOsteosarcoma connects-toNeuron connects-toFibroblast connects-toP53 connects-toProton connects-toNervous System connects-toVegf connects-toMtor connects-toMacrophage connects-toNeurofibromatosis Type 2 connects-toKras connects-toCdk4 6 connects-toNatural Killer Cell connects-toLung connects-toOxygen connects-toT Cytotoxic Cell connects-toLiver connects-toEndothelial Cell connects-toFibrosis connects-toElectron connects-toIgf 1 connects-toAkt connects-toBone Marrow connects-toBrain connects-toLarge Intestine connects-toAntibody connects-toNeutrophil connects-toSkin connects-toCardiomyocyte connects-toReproductive System connects-toPlatelet connects-toEgfr connects-toRegulatory T Cell connects-toMelanoma connects-toNeuropathic Pain connects-toPdgf connects-toStat3 connects-toNf Kb connects-toVenous Thromboembolism connects-toSepsis connects-toHeart Failure connects-toAspergillus Fumigatus connects-toMajor Depressive Disorder connects-toCkd connects-toWound Healing connects-toGeneralized Anxiety Disorder connects-toRespiratory System connects-toIntegumentary System connects-toVaricella Zoster Virus connects-toRenal System connects-toCardiovascular System connects-toLymphatic System connects-toDigestive System connects-toImmune System connects-toTargeted Therapy connects-toCancer Chemotherapy connects-toNeuroblastoma connects-toChordoma connects-toPheochromocytoma Paraganglioma connects-toCheckpoint Inhibitors connects-toCmml connects-toDiffuse Midline Glioma connects-toAlveolus connects-toAxonal Transport connects-toDesmoid Tumor connects-toGlioblastoma connects-toH3K27M connects-toMyc connects-toTert connects-toBraf connects-toCyclin D1 connects-toMet connects-toHif 1Alpha connects-toRb1 connects-toCcl2 connects-toCaspase 3 connects-toCxcl12 connects-toKit connects-toPten connects-toDnmt3A connects-toPerforin connects-toRad51 connects-toE2F1 connects-toPik3Ca connects-toMdm2 connects-toJak1 2 connects-toBcl 2 connects-toNfe2L2 connects-toTgf Beta connects-toGalectin 3 connects-toCgas Sting connects-toStat1 connects-toSmad4 connects-toFoxo connects-toGsk 3B connects-toS100A8 A9 connects-toSrc Kinase connects-toAutophagy connects-toArid1A connects-toAmpk connects-toCcr5 connects-toIl 6 connects-toYap1 connects-toIl 1B connects-toIl 33 connects-toIl 17A connects-toComplement C3 connects-toCalcineurin connects-toOsteopontin connects-toMhc Class Ii connects-toIl 2 connects-toPd 1 connects-toHemoglobin connects-toTroponin Complex connects-toIl 10 connects-toIl 4 connects-toXanthine Oxidase connects-toNitric Oxide connects-toIl 13 connects-toIron connects-toCortical Bone connects-toLeptin connects-toAdiponectin connects-toResistin connects-toType I Interferon connects-toIfn Gamma connects-toIl 12 connects-toIl 5 connects-toIl 23 connects-toIge connects-toMast Cell connects-toT Helper Cell connects-toDendritic Cell connects-toB Cell connects-toC5Ar1 connects-toComplement C5 connects-toTslp connects-toHistamine connects-toBradykinin connects-toC1 Esterase Inhibitor connects-toErythropoietin connects-toPeriostin connects-toMelatonin connects-toTestosterone connects-toSerotonin connects-toProlactin connects-toOxytocin connects-toVasopressin connects-toSelenium connects-toIodine connects-toSodium connects-toMagnesium connects-toCopper connects-toZinc connects-toCalcium connects-toCarbon connects-toNitrogen connects-toChloride connects-toHydrogen connects-toPhosphorus connects-toGlp 1 connects-toAngiotensin Ii connects-toWnt Beta Catenin connects-toRankl connects-toFibronectin connects-toActivin A connects-toTgf Beta connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toNotch connects-toAndrogen Receptor connects-toNorepinephrine connects-toAdrenomedullin connects-toBdnf connects-toFgfr

Sources