bösartiger peripherer Nervenscheidentumor (MPNST)
Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.
| ID | DIS-MPNST |
|---|---|
| Art | Erkrankung |
| Aliase | MPNSTbösartiger peripherer NervenscheidentumorMalignant schwannoma (historische)Neurofibrosarkom (historische)Злоякісна пухлина оболонки периферичного нерва |
| Status | reviewed 2026-04-27 | pending_clinical_signoff |
| Erkrankungen | None declared |
| Quellen | 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 Weichgewebe Tumore 5 ed (2020): Tumor der bösartigen peripheren Nervenscheide |
Anmerkungen
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...
Verwendet von
Algorithmen
ALGO-MPNST-1L- ALGO-MPNST-1L
Erkrankung
DIS-EPITHELIOID-SARCOMA- Epithelioidsarkom
Anwendungsgebiet
IND-MPNST-DOXORUBICIN-IFOSFAMIDE- IND-MPNST-DOXORUBICIN-IFOSFAMIDE
Fragebogen
QUEST-MPNST-1L-STUB- bösartiger peripherer Nervenscheidentumor — erste Zeile
Warnhinweis
RF-MPNST-ADVANCED-DOXORUBICIN-UNSUITABLE- Fortgeschritten/metastastisch MPNST mit LVEF <50% OR kumulatives Anthracyclin ≥350 mg/m² OR ECOG ≥...RF-MPNST-FRAILTY-AGE- Alter ≥75 mit ECOG ≥2 oder ≥2 Comorbiditäten — Doxorubicin + ifosfamide (AI) neoadjuvanten Armen...RF-MPNST-HIGH-RISK-BIOLOGY- MPNST with adverse molecular / clinical features: biallelic CDKN2A/B loss (>80% high-grad...RF-MPNST-INFECTION-SCREENING- Aktive oder latente Infektion, die eine Auflösung / Prophylaxe vor Beginn der Doxorubie erfordert...RF-MPNST-ORGAN-DYSFUNCTION- Ausgangsorgan Dysfunktion, die Standard Doxorubicin + ifosfamide (AI) neoadjuvant...RF-MPNST-TRANSFORMATION-PROGRESSION- Atypisches neurofibromatöses Neoplasma mit unsicherem biologischem Potential (ANNUBP) Progression...
Prüfer
REV-DENISOVA-OLGA- {"english": "Olga Denisova", "bevorzugt": "Olga Denisova", "Suffix": "MD", "ukrainisch": "...