Rhabdomyosarcoma PDQ®

In brief

Comprehensive NCI clinical reference for healthcare professionals managing childhood rhabdomyosarcoma, the most common soft tissue sarcoma in children. Covers evidence-based treatment protocols including multimodal therapy with surgery, chemotherapy, and radiation, plus risk stratification and prognosis.

• Rhabdomyosarcoma is the most common soft tissue sarcoma in children, arising from primitive mesenchymal cells
• Risk stratification depends on histology (embryonal vs alveolar), primary site, tumor size, nodal status, and metastases
• Multimodal therapy combining surgery, chemotherapy, and radiation achieves 5-year survival >70% in localized disease
• Alveolar subtype with PAX-FOXO1 fusion carries worse prognosis and requires intensified chemotherapy protocols
• Treatment aims to balance cure with minimizing long-term toxicity; organ preservation is prioritized when oncologically safe

Written by the GCMD Library team from the guideline.