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rhabdomyosarcoma
Rhabdomyosarcoma
Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma; embryonal (~60%, VAC backbone, 5-year FFS ~90% low-risk) and alveolar (~25%, PAX3/PAX7-FOXO1 fusions, high-risk) subtypes; VAC±irinotecan; RT; metastatic disease 5-year OS ~20-30%.
Entry Metadata
| Field | Value |
|---|---|
| ID | rhabdomyosarcoma |
| Name | Rhabdomyosarcoma |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 07-system |
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Sources
- Crist WM, Anderson JR, Meza JL, et al. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol. 2001;19(12):3091-3102. · PubMed 11408506
- Oberlin O, Rey A, Sanchez de Toledo J, et al. Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas. J Clin Oncol. 2012;30(19):2457-2465. · PubMed 22665546