Occurring at a deeper level than most other cancers, sarcomas are often detected late and at an advanced stage of development. Since they arise from a range of different tissue-types, sarcomas vary widely in form. They can affect people of all ages but are generally rare, accounting for only 1% of all cancer diagnoses in the UK – around 2,400 cases a year.
Sarcoma often requires radical surgical treatment and should be carried out in specialist units. Treatment is bespoke and tailored to a patient’s circumstances. Prognosis can be good providing that surgery is timely and thorough.
What does this condition involve?
As sarcoma affects different people in different places, it is difficult to provide a clear single picture of the condition – or a patient’s experience of it. About 55% of sarcomas affect a patient’s limbs, most frequently the leg; about 15% affect the head, neck or trunk, while the remainder occur in the abdominal region.
Sarcomas fall into three broad categories – soft tissue sarcomas, primary bone cancers or osteosarcomas, and gastro-intestinal stromal tumours (or GIST), which is the most common. The soft tissue sarcomas tend to be named according to their tissue of origin, for example, sarcomas arising from cartilage are known as chondrosarcomas, while those that grow out of smooth muscle are referred to as leiomyosarcomas.
The causes of most sarcomas are unknown. Some hereditary syndromes carry a pre-disposition to sarcoma, but the number of these cases is very small. Patients who have had a certain form of eye cancer in childhood may also have a propensity for sarcoma, while there is evidence that prolonged or extensive exposure to certain chemicals may also be a cause. Occasionally, sarcoma diagnosis can be related to the use of radiotherapy to treat other forms of cancer.
Sarcomas usually manifest themselves as slow-growing lumps, a little bigger than a golf ball. If you notice anything like this anywhere on your body, you should seek medical advice.
What surgery is available, and what techniques are involved?
In order to cure sarcoma, wide local excision surgery is required. This means not only removing the tumour, but cutting out a margin of surrounding tissue also. Surgeons will look to achieve clear margins of surrounding tissue, and these margins will then be analysed in a pathology lab.
If it is clear that the disease has spread beyond the tumour itself, radiotherapy may be proposed. For the treatment of bone sarcomas, pre and post-surgery chemotherapy is often used, but this is not recommended for soft tissue tumours. GIST sarcomas respond poorly to chemo or radiotherapy, but can be treated with a new drug called Glivec.
As with other cancers, excision surgery will be followed with reconstructive surgery in order to the fill the gap, or the deficit, left by the removal of the tumour. Depending on the extent of the deficit, a number of plastic surgery techniques can be employed, but in the main these will involve flap surgery.
A local flap is a piece of living tissue that is transferred from one part of the body to an adjacent area along with the blood vessels that keep it alive. Free flap surgery is similar in concept, but unlike local flap transfer, free flaps are entirely disconnected from their original blood supply and are reconnected using microsurgery in the recipient site. The main benefit of free flap surgery is that surgeons can tailor the reconstruction very precisely to a patient’s needs. Depending on the nature of the defect caused by the surgical excision, surgeons can make free flaps either thin and pliable, or bulky and padded. The free flap technique also enables surgeons to reduce what is called donor site morbidity – which means reducing the damage caused to the area from where the free flap tissue has been taken.
Is this surgery available on the NHS?
The surgical treatment of sarcoma is available on the NHS and should be carried out in designated sarcoma centres.
Who will I see as patient?
In 2006, guidance published by the National Institute of Clinical Excellence (NICE) mandated that all patients with sarcoma should be treated in specialist units. In these sarcoma units, patients are seen by multi-disciplinary teams. A multi-disciplinary sarcoma team will be made up of specialists working together to make sure that the best possible treatment is given.
These specialists should include the following:
• Clinical specialist nurse
• Oncological surgeon
• Plastic surgeon
As sarcomas occur in different parts of the body, surgeons from other disciplines may be called in to conduct certain specialist procedures. For example, if a patient is suffering from a spinal sarcoma, a spinal surgeon surgeon may be called upon to help treat that particular patient.
What should I expect in terms of treatment, procedures and outcomes?
While sarcoma is an unusual form of cancer, swift and aggressive surgical treatment can lead to good results. Reconstructive surgeons will do all they can to restore form and function in the affected area. When assessing the options for reconstruction, sensible clinical decisions will be made to give the patient the best possible chance of a good functional and aesthetic outcome.
For sarcoma patients undergoing excision and reconstructive surgery, tissues should be healed within a few weeks. For patients requiring complex surgery on the chest wall or lower limb, the recovery time can be a lot longer.
When a patient is suffering from sarcoma in the arm or leg, limb salvage will be the surgeon’s first priority, although amputation can occasionally be the most definitive treatment option available. With the advancements in prosthetic limb technology, amputation – which should not be regarded as a surgical failure – now carry a good prospect of functional recovery.
NICE – Sarcoma
Macmillan Cancer Support – information on soft tissue sarcomas
Macmillan Cancer Support – information on Kaposi’s sarcoma
National Cancer Institute – information on soft tissue sarcomas
Cancer Research UK – information on soft tissue sarcomas
Children’s Cancer Web – information on Ewing’s sarcoma
NHS Choices – information on Kaposi’s sarcoma
NHS Choices – information on bone sarcoma