Epidemiology
Most common soft tissue sarcoma in children and adolescents (< 18 years) (50%)
Types
Heterogenous group with myogenic differentiation
80% embyonal / alveolar
Embryonal (50%)
- balanced chromosomal translocations t(2:13)
- expression of the fusion oncoproteins PAX3-FOXO1 or PAX7-FOXO1
- most common in children (Embryonal = Early)
- nasal cavity, orbit, middle ear, prostate, testes
Alveolar (30%)
- worse outcomes than embryonal
- head and neck / proximal extremity lesions
Pleomorphic (~20%)
- more common in adults
- 30% survival
- proximal extremity lesion
Spindle cell
MRI
Pleomorphic rhabdomyosarcoma adult
Management
Principles
Chemotherapy
- rhabdomyosarcoma is chemosensitive
- 70 - 80% response rate
- neoadjuvant / adjuvant
Wide surgical resection
Radiotherapy
Prognosis
Can spread to nodes, atypical for most sarcomas
Better prognosis in children, compared to adults
Bisogno et al Lancet Oncol 2019
- 371 patients with high risk rhabdomyosarcoma
- 5 year survival with maintenance chemo: 87%
- 5 year survival without maintenance chemo: 74%
- age 0 - 14: 5 year survival 67%
- age 15 - 19: 5 year survival 40%