Soft tissue sarcomas (clinicians)
About soft tissue sarcomas
Soft tissue sarcomas are named depending on the type of tissue they develop on. In the UK, over 200 children and young people are diagnosed each year.
The most common soft tissue sarcoma is rhabdomyosarcoma with around 60 children diagnosed annually.
Different types of sarcomas include:
- Rhabdomyosarcoma in skeletal muscle
- Fibrosarcoma in fibrous tissues such as tendons and ligaments
- Leiomyosarcoma, in smooth muscle such as the stomach and bladder
- Schwannoma in peripheral nerve sheaths (peripheral nerve sheath tumour)
- Liposarcoma, in fat cells
- Primitive neuroectodermal tumours (pPNETs) in cells of neuroectodermal origin
Signs & symptoms
- Swelling or lump – often these may occur on the head, neck, testes or vagina. Initially, they are often painless
- Nasal blockage or discharge (facial tumour)
- Haematuria
- Change in bowel habit, especially constipation
- Tiredness
- Pallor
Rhabdomyosarcoma
The most common location for rhabdomyosarcoma is in the head and neck, but other common sites are the bladder and the testes. It is however possible for it to be found anywhere in the body. The most common sign is a swelling or lump.
There are a number of syndromes that result in an increased risk of rhabdomyosarcoma occurring:
- Li-Fraumeni syndrome (type 1)
- Neurofibromatosis
- Beckwith-Wiedemann syndrome
- Costello syndrome
- Noonan syndrome
Treatment
Usually, a combination of chemotherapy, radiotherapy and surgery is likely to be used. Depending on the stage at diagnosis, the treatment is quite aggressive. Some treatment options include chemotherapy as maintenance treatment.
Prognosis
Rhabdosarcoma has a 5-year survival of 71%. The most important prognostic factor is the age of the child at diagnosis. Younger children on average have much better outcomes than older children.
If a child is over 10 years old at diagnosis, their five-year survival rates drop to around 50%.
