Infantile fibrosarcoma (IFS) is defined by ETV6-NTRK3 fusion (>90%). Larotrectinib has tr...
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| ID | BMA-NTRK-FUSION-IFS |
|---|---|
| Tipo | Relevância terapêutica |
| Estado | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Doenças | DIS-IFS |
| Fontes | SRC-CIVIC SRC-FDA-CDS-2026 |
Fatos de ação
| Biomarcador | BIO-NTRK-FUSION |
|---|---|
| Variante | Fusão ETV6-NTRK3 (lesão definida) |
| Doença | DIS-IFS |
| Nível ESCAT | IA |
| Combinações recomendadas | larotrectinib monotherapy (1L for unresectable / metastatic IFS — chemotherapy-sparing), entrectinib monotherapy |
| Contraindicated monotherapy | VAC chemotherapy (vincristine/actinomycin/cyclophosphamide) as 1L when NTRK-TKI available — no longer standard given larotrectinib tolerability and efficacy |
| Resumo das provas | Infantile fibrosarcoma (IFS) is defined by ETV6-NTRK3 fusion (>90%). Larotrectinib has transformative activity (SCOUT pediatric trial, Laetsch Lancet Oncol 2018 — ORR 93% in IFS), enabling organ-sparing surgery and avoiding mutilating resection or intensive chemotherapy. Tumor-agnostic FDA approval since 2018; SCOUT data drove pediatric label. |
Notas
ESCAT IA. OncoKB Level 1. IFS is the prototype tumor for the paradigm shift from cytotoxic to targeted therapy in pediatric oncology. Prior standard (VAC chemotherapy) achieved ~70% response but with substantial toxicity. Larotrectinib enables less mutilating surgery in localized IFS. Source-gap: SRC-NCCN-PEDIATRIC-SARCOMA / SRC-SCOUT not yet ingested.
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