Soft tissue sarcomata are rare tumours accounting for less than 1% of adult tumours but up to 6% of childhood tumours.
A genetic risk factor for sarcoma formation may be present in as many as one third of all patients with childhood sarcomata.
Familial syndromes causing sarcomata include:
- familial retinoblastoma
- Li-Fraumeni syndrome
- Beckwith-Wiedemann syndrome
- basal cell naevus
- Gardner syndrome