The most common soft tissue sarcoma in children, arising from skeletal muscle progenitors. Two main types: embryonal (including botryoid and spindle cell variants) and alveolar. Can occur anywhere in body including head/neck, genitourinary, and extremities.

• 1Biopsy
• 2Imaging (CT, MRI, PET)
• 3Tumor markers
• 4Genetic testing
• 5Endoscopy
• 6Blood tests
• 7Screening programs

Localized disease has 70-90% long-term survival depending on histology and site. Embryonal histology has better prognosis than alveolar. Orbit and non-parameningeal head/neck sites have best outcomes (>90%). Parameningeal and extremity sites have poorer prognosis. Metastatic disease at diagnosis has 20-30% survival. IRS/COG staging guides intensity of therapy. Late effects include growth delay, infertility, and second cancers. Long-term surveillance essential.

• Smoking cessation
• Sun protection
• Healthy diet
• Regular exercise
• Vaccination (HPV, HBV)
• Screening programs
• Limit alcohol
• Maintain healthy weight