Malignant peripheral nerve sheath tumor (MPNST)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-MPNST |
|---|---|
| Type | Disease |
| Aliases | MPNSTMalignant peripheral nerve sheath tumorMalignant schwannoma (historical)Neurofibrosarcoma (historical)Злоякісна пухлина оболонки периферичного нерва |
| Status | reviewed 2026-04-27 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-NCCN-NSCLC-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9540/3 |
|---|---|
| icd_o_3_topography | ["C47"] |
| icd_10 | C47 |
| who_classification | WHO Soft Tissue Tumours 5th ed (2020): Malignant peripheral nerve sheath tumour |
Notes
Aggressive soft-tissue sarcoma arising from peripheral nerve sheath Schwann cells. Most surgically treatable when diagnosed early; once metastatic, systemic options are limited. Standard treatment: - Localized: wide en-bloc surgical resection ± adjuvant RT. Chemotherapy (doxorubicin + ifosfamide) for high-grade per soft- tissue sarcoma protocols; benefit modest. - Metastatic: doxorubicin ± ifosfamide (1L); pazopanib (PALETTE); trabectedin / eribulin in later lines. Targeted-therapy investigational signals (no FDA approval to date for MPNST specifically): - MEK inhibitors (selumetinib, mirdametinib): rationale from NF1 → loss of neurofibromin → unrestrained RAS → MAPK; trials ongoing in NF1-related MPNST. Selumetinib is FDA-approved for NF1-associated symptomatic inoperable plexiform NEUROFIBROMA (SPRINT trial) — the pre-malignant precursor — but not yet for MPNST proper. - SHP2 inhibitors: investigational. - CDK4/6 inhibitors (CDKN2A loss → synthetic-lethality hypothesis): investigational. - Mirdametinib + selumetinib + similar agents being studied in NF1 consortia for atypical neurofibroma → MPNST transition. Surveillance: NF1 patients with rapidly enlarging or painful neurofibro...
Used By
Algorithms
ALGO-MPNST-1L- ALGO-MPNST-1L
Disease
DIS-EPITHELIOID-SARCOMA- Epithelioid sarcoma
Indication
IND-MPNST-DOXORUBICIN-IFOSFAMIDE- IND-MPNST-DOXORUBICIN-IFOSFAMIDE
Questionnaires
QUEST-MPNST-1L-STUB- Malignant peripheral nerve sheath tumor — first line
Red flag
RF-MPNST-ADVANCED-DOXORUBICIN-UNSUITABLE- Advanced/metastatic MPNST with LVEF <50% OR cumulative anthracycline ≥350 mg/m² OR ECOG ≥...RF-MPNST-FRAILTY-AGE- Age ≥75 with ECOG ≥2 or ≥2 comorbidities — doxorubicin + ifosfamide (AI) neoadjuvant poor...RF-MPNST-HIGH-RISK-BIOLOGY- MPNST with adverse molecular / clinical features: biallelic CDKN2A/B loss (>80% high-grad...RF-MPNST-INFECTION-SCREENING- Active or latent infection requiring resolution / prophylaxis before initiating doxorubic...RF-MPNST-ORGAN-DYSFUNCTION- Baseline organ dysfunction precluding standard doxorubicin + ifosfamide (AI) neoadjuvant...RF-MPNST-TRANSFORMATION-PROGRESSION- Atypical neurofibromatous neoplasm of uncertain biologic potential (ANNUBP) progression t...
Reviewers
REV-DENISOVA-OLGA- {"english": "Olga Denisova", "preferred": "Olga Denisova", "suffix": "MD", "ukrainian": "...