Rhabdomyosarcoma

 

Thigh sarcomaSarcoma Thigh Axial

 

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

 

Rhabo adult Rhabdo adult 2

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%

 

Trama et al Lancet Oncol 2016

- age 0 - 14:  5 year survival 67%

- age 15 - 19: 5 year survival 40%