Treatment and care of people with cancer is usually provided by a team of health professionals, both medical and allied health, called a multidisciplinary team.

Treatment for sarcoma depends on:

• the type of sarcoma
• where it is in the body
• whether the cancer has spread, and where it has spread to
• your general health
• your personal preferences.

Treatment options can include surgery, chemotherapy, radiation therapy 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 areas of 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.

Gastrointestinal stromal tumours (GIST) very rarely spread to the lymph nodes, so it is usually not necessary to remove nearby lymph nodes for this type of sarcoma.

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 arm or leg might need to be amputated (partially or completely removed), but this is not common. The cancer can usually be removed without amputation.

Chemotherapy

Along with surgery, some people may receive chemotherapy.

Chemotherapy may be used to make the tumour smaller before surgery or 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 is not commonly used to treat gastrointestinal stromal tumours (GIST) because these tumours rarely shrink in response to these types of medicines.

Radiation therapy

Along with surgery, some people may receive radiation therapy.

Radiation therapy may be used to make the tumour smaller before surgery or to remove any remaining cancer cells after surgery.

Radiation therapy is not very helpful for treating gastrointestinal stromal tumours (GIST), 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. Targeted therapies for soft tissue sarcoma and gastrointestinal stromal tumours (GIST) may include:

• monoclonal antibodies that target proteins on cancer cells to kill them or block their growth
• tyrosine kinase inhibitors, which block enzymes that are important for growth and survival of cancer cells
• mTOR inhibitors, which block a protein called mTOR that stops cancer cells from growing and stops new blood vessels forming in the cancer.

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 radiation therapy.

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, radiation therapy or targeted therapy.

Another treatment option for recurrent 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.

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

Treatment of recurrent gastrointestinal stromal tumours (GIST) might involve further surgery or targeted therapy.