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
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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

Sources