Rhabdomyosarcoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-RHABDOMYOSARCOMA |
|---|---|
| Type | Disease |
| Aliases | Alveolar rhabdomyosarcoma (ARMS)Embryonal rhabdomyosarcoma (ERMS)RMSSarcoma of skeletal muscle differentiationРабдоміосаркома |
| Status | reviewed 2026-05-19 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | 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) |
Notes
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.
Used By
Disease
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