Key Facts
Sarcomas are tumours that develop from the supporting tissues in the body which include the bones, muscles, fat, blood vessels, nerves and joints.
All sarcomas are very rare. Overall, sarcomas account for just 1% of all cancers.
Sarcomas often go undiagnosed. Any swelling in the soft tissues greater than 5cm in size could be a sarcoma and should be referred to a specialist centre for investigation.
Cells in sarcomas often look like cells from cartilage, fat or muscle.
The treatment for the different types of sarcoma varies according to the type of tumour, growth rate and site.
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Key Figures
Sarcomas account for just 1% of all cancers.
Incidence and response to treatment vary enormously according to the type of tumour.
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Cancer Types
Muscle Cell Sarcomas
Muscle cell sarcomas are very rare. There are two main types of muscle cell sarcoma:
Bone Cell Sarcomas
Bone sarcomas are tumours which arise in the first place from bone cells and must be distinguished from secondary cancers which have spread to the bones from primary sites such as lung, breast and prostate.
Osteosarcoma
Osteosarcomas mainly affect the young and the very old. In children, teenagers and young adults the most likely symptoms are a persistent pain or a bony lump. In elderly people, osteosarcomas can develop from Paget's disease and are often noticed following a fall.
Paget's disease of the bone affects people over the age of 40. It is found more often in men than women and occurs most commonly in the UK.
Chondrosarcoma
The cells of chondrosarcomas resemble cartilage tissue cells. They are found in the thigh, arm, pelvis, knee and spine.
Ewing's Sarcoma
Ewing's sarcoma is the name given to a very rare group of sarcomas which affect teenagers. Ewing’s sarcoma usually starts in the bone – most commonly of the pelvis, femur (thigh bone) or tibia (shin bone).
GastroIntestinal Stromal Tumours (GIST)
GastroIntestinal Stromal Tumours (GIST) are rare tumours of the digestive system (1-3% of all gastrointestinal malignancies) and are a form of sarcoma. 70% occur in the stomach and they can spread to the liver and elsewhere within the abdominal cavity.
Malignant Fibrous Histiocytoma (MFH)
Malignant Fibrous Histiocytoma (MFH) is an uncommon form of sarcoma which is found in the fibrous tissue in and around the bones.
Fatty Tissue Sarcomas
Liposarcomas have cells that resemble fat cells. There are three main types of liposarcoma:
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Causes of Sarcomas
In most cases, there is no clear reason for a sarcoma to have developed. For most patients, therefore, there are no risk factors to avoid.
However, osteosarcomas may develop in patients with Paget’s disease.
People with rare inherited genetic conditions, such as neurofibromatosis, Gardner syndrome and retinoblastoma, are at greater risk.
Sarcomas can also very rarely occur 5–10 years or more after a patient has received radiotherapy for another type of cancer.
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Prevention of Sarcomas
In most cases, there is no clear reason for a sarcoma to have developed. For most patients, therefore, there are no risk factors to avoid.
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Early Detection
Awareness is critically important for early diagnosis. Any swelling in the soft tissues greater than 5cm in size could be a sarcoma and should be referred to a specialist centre for investigation. In practice, as many as 10% of all swellings of this size are sarcomas.
Patients with neurofibromatosis, Gardner syndrome and retinoblastoma should undergo regular check-ups.
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Common Symptoms
Pain and swelling are the commonest presenting problems although fractures through tumours may also occur and there may be symptoms of fever and weight loss with Ewing's sarcoma.
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Diagnostic Tests
Isotope bone scans, MRI and CT scans of the tumours are helpful in suggesting the diagnosis which is confirmed by biopsy.
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Treatment
Successful treatment of sarcomas requires a specialist team of health professionals.
Surgery, radiotherapy, chemotherapy and, less commonly, the newer targeted therapies (monoclonal antibodies) are all used to treat sarcomas. Usually more than one form of treatment will be required.
Using the combined knowledge of all the multidisciplinary team members, these different treatments can be combined to produce better results than any one treatment given on its own.
The recommended treatment will depend on the type of tumour, where it is, whether it has spread to other areas, and the patient’s own preferences once the options for treatment have been explained.
The aim of surgery is to remove all or as much of the tumour as possible. Usually, the cancer is removed with a margin of normal tissue around it so that there is the maximum chance of removing all the malignant cells. This reduces the risk of the sarcoma coming back in the same place.
Using radiotherapy, chemotherapy or both (chemoradiation) before surgery can in some cases reduce the size of the tumour and make surgery easier.
Radiotherapy can also be used as an alternative to surgery for patients for whom surgery is not feasible.
In the past, sarcomas of the arms and legs were treated by amputation. However, it is now usually possible to remove just the cancer and a safe margin of tissue around it - particularly if further chemotherapy or radiotherapy is used.
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Key Trends
- Treatments for sarcomas are constantly developing.
- Sarcomas are (and should be) treated in specialist units.
- Use of the new targeted therapies (monoclonal antibodies) is an important aspect of research for many different types of sarcoma.
- The quality of surgery is increasingly regarded as essential in securing good results.
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