Infantile fibrosarcoma (IFS)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-IFS |
|---|---|
| Type | Disease |
| Aliases | Congenital fibrosarcomaInfantile fibrosarcomaІнфантильна фібросаркома |
| Status | reviewed 2026-04-27 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | 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 | 8814/3 |
|---|---|
| icd_o_3_topography | ["C49"] |
| icd_10 | C49 |
| who_classification | WHO Soft Tissue Tumours 5th ed (2020): Infantile fibrosarcoma (NTRK-rearranged spindle cell neoplasm — pediatric) |
Notes
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.
Used By
Actionability
BMA-NTRK-FUSION-IFS- Infantile fibrosarcoma (IFS) is defined by ETV6-NTRK3 fusion (>90%). Larotrectinib has tr...
Algorithms
ALGO-IFS-1L- ALGO-IFS-1L
Indication
IND-IFS-NTRK-LAROTRECTINIB- IND-IFS-NTRK-LAROTRECTINIB
Questionnaires
QUEST-IFS-1L-STUB- Infantile fibrosarcoma — first line
Red flag
RF-IFS-FRAILTY-AGE- Pediatric IFS with neonatal age (<3 months), low birth weight / failure to thrive (weight...RF-IFS-HIGH-RISK-BIOLOGY- Infantile fibrosarcoma with NTRK fusion identified (ETV6-NTRK3 in >90%, LMNA-NTRK1 / TPM3...RF-IFS-INFECTION-SCREENING- Active or latent infection requiring resolution / prophylaxis before initiating VAC cytot...RF-IFS-ORGAN-DYSFUNCTION- Baseline organ dysfunction in pediatric IFS precluding standard VAC (vincristine / actino...RF-IFS-TRANSFORMATION-PROGRESSION- Progression of infantile fibrosarcoma on first-line larotrectinib / entrectinib (acquired...