Tumor maligno de la cubierta del nervio periférico (MPNST)
Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.
| ID | DIS-MPNST |
|---|---|
| Tipo | Enfermedad |
| Aliaces | MPNSTTumor maligno de la vaina del nervio periféricoEscwannoma maligno (histórico)Neurofibrosarcoma (histórico)Злоякісна пухлина оболонки периферичного нерва |
| Situación | reviewed 2026-04-27 | pending_clinical_signoff |
| Enfermedades | Ninguna declarada |
| Fuentes | 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 |
Notas
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...
Utilizado por
Algoritmos
ALGO-MPNST-1L- ALGO-MPNST-1L
Enfermedad
DIS-EPITHELIOID-SARCOMA- Sarcoma epitelioide
Indicación
IND-MPNST-DOXORUBICIN-IFOSFAMIDE- IND-MPNST-DOXORUBICIN-IFOSFAMIDE
Cuestionarios
QUEST-MPNST-1L-STUB- Tumor maligno del nervio periférico en la vaina — primera línea
Señal de alerta
RF-MPNST-ADVANCED-DOXORUBICIN-UNSUITABLE- Avanzado/metástatico MPNST con LVEF <50% OR acumulación de antraciclina ≥350 mg/m² OR ECOG ≥...RF-MPNST-FRAILTY-AGE- Edad ≥75 con ECOG ≥2 o bien ≥2 Comorbidades — doxorrubicina + ifosfamide (AI- Neoadyuvante pobre...RF-MPNST-HIGH-RISK-BIOLOGY- MPNST con características moleculares / clínicas adversas: pérdida de CDKN2A/B biallélica (>80% de alto grado...RF-MPNST-INFECTION-SCREENING- Infección activa o latente que requiere resolución / profilaxis antes de iniciar doxorúbica...RF-MPNST-ORGAN-DYSFUNCTION- Disfunción de los órganos basales que excluye doxorrubicina estándar + ifosfamide (AI) neoadyuvante...RF-MPNST-TRANSFORMATION-PROGRESSION- Neoplasia neurofibromatosa atípica de progresión incierta del potencial biológico (ANNUBP)
Revisores
REV-DENISOVA-OLGA- {"english": "Olga Denisova", "preferido": "Olga Denisova", "sufijo": "MD", "ukrainian": "...