Onco Wiki / Doença

Fibrossarcoma infantil (IFS)

Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.

IDDIS-IFS
TipoDoença
Alegais
Fibrossarcoma congénitoFibrossarcoma infantilІнфантильна фібросаркома
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_morphology8814/3
icd_o_3_topography["C49"]
icd_10C49
who_classificationWHO Soft Tissue Tumours 5th ed (2020): Infantile fibrosarcoma (NTRK-rearranged spindle cell neoplasm — pediatric)

Capacidades

Notas

Defining indication for tumor-agnostic NTRK-targeted therapy in pediatric oncology — historical care was VAC chemotherapy (vincristine + actinomycin + cyclophosphamide) ± surgery, with morbidity from large surgical resections in young infants. Therapy: - Larotrectinib (SCOUT pediatric arm): ORR ~94% in NTRK-fusion+ IFS; used as neoadjuvant to convert unresectable / morbid resections to function-preserving surgery. - Entrectinib (STARTRK-NG): similar approach. - Surgery: complete resection if achievable without major morbidity; otherwise neoadjuvant TRK inhibitor → re-evaluate resection. - Chemotherapy (VAC): reserved for fusion-negative or TRK-resistant cases. Resistance: solvent-front (G623R for NTRK3; G595R for NTRK1) and gatekeeper mutations on progression — selitrectinib / repotrectinib in development. Pediatric cardiac / neurodevelopmental long-term monitoring on TRK inhibitors is an active research question.

Usado por

Relevância terapêutica

Algoritmos

Indicação

Questionários

Sinal de alerta