Atlas One · Host Biology · Scale 07 — System

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%.

Also known as: rhabdomyosarcoma, RMS, embryonal RMS, alveolar RMS, ERMS, ARMS, pediatric soft tissue sarcoma, botryoid RMS

Scale 07 — SystemScale
draftStatus
2026-06-06Last Reviewed
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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

FieldValue
IDrhabdomyosarcoma
NameRhabdomyosarcoma
Statusdraft
Last reviewed2026-06-06
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toFoxo1 connects-toMyc connects-toAkt connects-toMet connects-toLi Fraumeni Syndrome connects-toIgf 1 connects-toDicer1 connects-toWilms Tumor connects-toMusculoskeletal System connects-toNeuroblastoma

Sources