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Tumor maligno da bainha do nervo periférico (MPNST)

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IDDIS-MPNST
TipoDoença
Alegais
MPNSTTumor maligno de bainha do nervo periféricoEsquenoma maligno (histórico)Neurofibrosarcoma (histórico)Злоякісна пухлина оболонки периферичного нерва
Estadoreviewed 2026-04-27 | pending_clinical_signoff
DoençasNenhuma declaração
FontesSRC-NCCN-NSCLC-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Fonte YAML

icd_o_3_morphology9540/3
icd_o_3_topography["C47"]
icd_10C47
who_classificationWHO Soft Tissue Tumours 5th ed (2020): Malignant peripheral nerve sheath tumour

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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...

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