Rabdomiossarcoma
Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.
| ID | DIS-RHABDOMYOSARCOMA |
|---|---|
| Tipo | Doença |
| Alegais | Rabdomiossarcoma alveolar (ARMS)Rabdomiossarcoma embrional (ERMS)RMSSarcoma de diferenciação muscular esqueléticaРабдоміосаркома |
| Estado | reviewed 2026-05-19 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-ESMO-SARCOMA-2024 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8910/3 |
|---|---|
| icd_o_3_topography | ["C49"] |
| icd_10 | C49.9 |
| who_classification | WHO Soft Tissue + Bone Tumours 5th ed (2020): Embryonal RMS, alveolar RMS, spindle-cell/sclerosing RMS, pleomorphic RMS (adult) |
Notas
PAX3-FOXO1 fusion status is the strongest single prognostic marker. EpSSG (European) and COG (North American) cooperatives study risk- adapted regimens. Fusion-positive ARMS now receives intensified treatment; fusion-negative ARMS behaves more like ERMS. Surveillance in Costello + LFS + DICER1 carriers: abdominal/pelvic US q3-6 mo to age 8-10; symptom-driven imaging for soft-tissue mass at any age.
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Doença
DIS-NEUROBLASTOMA- Neuroblastoma