Soft tissue sarcoma - further information

This objective of this page is to help you understand how sarcoma can develop, based on its 'natural history'.

Sarcomas frequently recur. Some recurrences will be local, rather than distant. These locally recurrent tumours can often be removed surgically, especially if they are on a limb, and the long term prognosis for such patients is no poorer as a result.

About 50% of sarcoma patients will have a recurrence involving metastasis, or secondary disease in a part of the body distant from the site of the primary tumour. The most usual site for metastasis is the lungs although the liver and, more rarely, bones or the lymph system, can be affected. Because many sarcomas are painless while they grow it is not uncommon for a patient to have disease which has advanced in this way when first diagnosed.

The first treatment option considered for metastasis will be surgery, if that is possible. As the most common metastatic site is the lungs the opinion of a specialist thoracic surgeon may be sought. However, many tumours will be unresectable or will be too fast-growing, numerous or widespread to allow surgery. Tumours can also recur again after a successful lung resection. In most cases the usual course of treatment will then be chemotherapy.

The two standard chemotherapy treatments available today are Doxorubicin and Ifosfamide. Response rates measured in clinical trials vary and trials comparing different dosages and patterns of dosage are still being undertaken.

Doxorubicin is usually administered as an out-patient. One of its side-effects is to weaken the heart so there is a lifetime limit on how much can be taken. Ifosfamide is usually given as an in-patient as it is now accepted that a high dose is the best approach. Ifosfamide is always given with Mesna, a drug which protects the bladder and kidneys from damage by the ifosfamide. The standard treatment pattern for both treatments is six cycles with treatment being repeated three weekly.

The latest approach is to use a combination of doxorubicin and high dose ifosfamide. This is a very toxic treatment and only suitable for someone who is relatively fit.

If you are considering chemotherapy ask the consultant or Clinical Nurse Specialist for any information they may have. There is also a very good information available from Macmillan (call 0808 808 00 00).

Sarcoma is regarded as an 'orphan' cancer, which means that it has few relationships with other more common cancers. It is also a rare disease and thus active research into new treatments is not regarded as a priority by many in the scientific or pharmaceutical world.

The innovative drug Yondelis (also known as trabectedin) has been approved for use in the NHS starting in 2010. This is a second-line drug, it can only be used after doxorubicin and ifosfamide. The drug has been developed from the toxin of a tiny sea squirt found in the Caribbean, although it is now synthesised by its manufacturer, the Spanish company Pharmamar.

Early stage clinical trials of new treatments are often undertaken at the principal specialist sarcoma centres.

Information on sarcoma clinical trials

Page reviewed December 2009