Treatment and care of people with cancer is usually provided by a team of health professionals - called a multidisciplinary team.

Treatment for sarcoma depends on:

• the type of sarcoma
• where it is in the body
• whether and how much the cancer has spread
• your general health and wishes.

Treatment options can include surgery, chemotherapy, radiotherapy and targeted therapy.

Surgery

Surgery is the most common treatment for soft tissue sarcoma and gastrointestinal stromal tumours in adults. The goal is to remove the entire tumour.

For soft tissue sarcomas, one type of surgery involves cutting the tumour from the skin in thin layers. The layers are examined under a microscope to check for cancer cells, and layers are removed until no more cancer cells are seen.

Another type of surgery involves wide local excision. This means removing the tumour along with some surrounding normal tissue.

If the tumour has spread to other sites in the body, surgery might still be used, but it might not be possible to remove all of the cancer using surgery alone. Lymph nodes might need to be removed if the cancer has spread to them. However, gastrointestinal stromal tumours very rarely spread to the lymph nodes, so it is usually not necessary to remove nearby lymph nodes for this type of sarcoma.

For gastrointestinal stromal tumours that have spread to the liver, ablation or embolisation might be used to destroy tumours. Ablation involves using extreme heat or cold, whereas embolisation involves injecting substances to try to block blood flow to cancer cells.

For sarcomas in bone, surgery will also be used to remove as much of the tumour as possible. For Ewing sarcoma, surgery is most often used after chemotherapy or radiotherapy to remove any cancer that is left.

In some cases of either soft tissue sarcoma or sarcoma in bone, the affected limb might need to be amputated, but this is not common. Usually, limb-sparing surgery can be used to remove the cancer without amputation.

Chemotherapy

Along with surgery, some people may receive chemotherapy.

Chemotherapy may be used before or after surgery to make the tumour smaller (before surgery) and remove any remaining cancer cells (after surgery).

Chemotherapy is part of the treatment for almost all patients with Ewing sarcoma. High-dose chemotherapy with a stem cell transplant may be used, especially for recurrent Ewing sarcoma. A stem cell transplant restores the body’s blood cells that were destroyed by the cancer treatment, using stem cells that were removed from the patient’s blood or bone marrow before treatment.

Chemotherapy using traditional chemotherapy medicines is not commonly used to treat gastrointestinal stromal tumours because these tumours rarely shrink in response to these types of medicines.

Radiotherapy

Along with surgery, some people may receive radiotherapy.

Radiotherapy may be used before or after surgery to make the tumour smaller (before surgery) and remove any remaining cancer cells (after surgery).

A treatment option for osteosarcoma is samarium. This is a radioactive substance that targets areas where bone cells are growing. Treatment with samarium may be followed by a stem cell transplant to restore the body’s blood cells that were destroyed by the cancer treatment.

Radiotherapy is not very helpful for treating gastrointestinal stromal tumours, but it is sometimes used to relieve symptoms such as pain.

Targeted therapy

Targeted therapy aims to destroy sarcoma cells while doing little damage to normal cells. One type of targeted therapy uses monoclonal antibodies that target proteins on cancer cells that can help them grow. The antibodies attach to the cancer cells and kill them or block their growth.

Another type of targeted therapy blocks enzymes called tyrosine kinases that are important for growth and survival of cancer cells.

Follow-up

After treatment, you might need regular physical examinations to check whether the cancer has come back (recurred). This might involve some of the tests that were done to diagnose the cancer.

Recurrent and secondary cancer

Sarcoma may recur (come back) after treatment. The cancer can recur at the same site or somewhere else in the body. Secondary cancer is when the cancer spreads to another part of the body.

For soft tissue sarcoma, treatment of recurrent disease might involve surgery, chemotherapy, or radiotherapy.

Treatment of recurrent osteosarcoma might involve further surgery, including surgery to remove cancer that has spread to other parts of the body, such as the lungs. It might also include chemotherapy, radiotherapy or targeted therapy.

Recurrent Ewing sarcoma might be treated using chemotherapy (including high-dose chemotherapy followed by a stem cell transplant), radiotherapy, surgery to remove tumours that have spread to the lungs, or targeted therapy.

Treatment of recurrent gastrointestinal stromal tumours might involve further surgery and/or targeted therapy.