Infantile fibrosarcoma (IFS) is defined by ETV6-NTRK3 fusion (>90%). Larotrectinib has tr...
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | BMA-NTRK-FUSION-IFS |
|---|---|
| Type | Actionability |
| Status | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Diseases | DIS-IFS |
| Sources | SRC-CIVIC SRC-FDA-CDS-2026 |
Actionability Facts
| Biomarker | BIO-NTRK-FUSION |
|---|---|
| Variant | ETV6-NTRK3 fusion (defining lesion) |
| Disease | DIS-IFS |
| ESCAT tier | IA |
| Recommended combinations | 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 |
| Evidence summary | 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. |
Notes
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.
Used By
No reverse references found in the YAML corpus.