Treatment

The treatment of sarcoma depends on the size and location of the tumor, its grade, its subtype, whether the cancer has spread, and the person’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.

This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.

Surgery is the most common treatment for sarcoma. If the tumor cannot be removed by surgery, it may be permanently controlled with radiation therapy. For a tumor that can be surgically removed, radiation therapy and/or chemotherapy may be given before or after surgery to reduce the risk of recurrence (cancer coming back after treatment). Chemotherapy and radiation therapy may also be used to reduce the size of the sarcoma or relieve pain and other symptoms.

Descriptions of the most common treatment options for sarcoma are listed below.

Surgery

Surgery is typically the main treatment for soft tissue sarcoma. A surgical oncologist is a doctor who specializes in treating cancer using surgery. The surgeon's goal is to remove the tumor and at least 1 cm to 3 cm (about 1 inch) of healthy tissue around it, to leave behind a clean margin. Small sarcomas can usually be cured by surgery alone, but those larger than 5 cm are usually treated with a combination of surgery and radiation therapy. Radiation therapy or chemotherapy may be used before surgery (to shrink the tumor and make removal easier), or during and after surgery (to kill any remaining cancer cells).

Learn more about cancer surgery.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor’s office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. Most chemotherapy drugs are given by injection into a vein (called intravenous or IV injection).

Some types of chemotherapy that might be used alone or in combination include doxorubicin (Adriamycin, Rubex), ifosfamide (Ifex), gemcitabine (Gemzar), docetaxel (Taxotere), dacarbazine (Matulane), paclitaxel (Taxol), as well as some targeted therapies (see below) called imatinib (Gleevec) (for GIST and a rare sarcoma called dermatofibrosarcoma protuberans), and sunitinib (Sutent; for GIST). Trabectedin (Yondelis) was recently approved in European countries for patients with sarcoma that continued to grow after initial chemotherapy. The specific drugs used to treat sarcoma depend on what subtype of sarcoma it is. Different drugs are used to treat different types and subtypes of sarcoma. Chemotherapy for sarcoma can usually be given as an outpatient treatment. Most side effects of the drugs go away within a short time after treatment.

Chemotherapy is often useful in cases in which a cancer has already metastasized. A fast-growing sarcoma can be treated with chemotherapy before surgery. This often reduces the size of the main tumor and may destroy tiny areas of metastasis if some of the cancer cells have already drifted into other areas.

For large high-grade sarcoma, where surgery may not be possible or is problematic, the doctor may recommend giving chemotherapy for three to four cycles before surgery to shrink the primary tumor, so it is more easily removable. Some chemotherapy before surgery may also improve survival since it may kill cells that have broken away from the original tumor. Chemotherapy may be given alone or in combination with surgery and/or radiation therapy. Chemotherapy given before surgery is called preoperative chemotherapy, neoadjuvant chemotherapy, or induction chemotherapy.

Chemotherapy given after surgery is called adjuvant chemotherapy or postoperative chemotherapy. After the patient has recovered from surgery, the oncologist may give more chemotherapy to kill any remaining tumor cells.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy.

Radiation therapy may be done before surgery to shrink the tumor, so it is more easily removed. Or, it may be done after surgery to remove any cancer cells left behind. Radiation treatment may make it possible to do less surgery, often preserving the arm or leg when that is where the sarcoma is located. Radiation therapy can also damage normal cells, but because it is focused around the tumor, side effects occur mainly in those areas. Most radiation therapy side effects go away soon after treatment ends. Newer radiation techniques, including intensity modulated radiation therapy and proton beam radiation (see Current Research), may help control sarcoma, as well as reduce the frequency of shorter-term and longer-term side effects.

Intensity modulated radiation therapy (IMRT). IMRT is a way to better direct radiation therapy at the tumor and reduce the risk of harming the bone or other nearby tissues.

Targeted therapy

Targeted therapy is a treatment that targets faulty genes or proteins that contribute to cancer growth and development. Therefore, it is much more specific for the treatment of the cancer, usually with fewer and less severe side effects. In 2002, the U.S. Food and Drug Administration (FDA) approved imatinib for the treatment of gastrointestinal stromal tumor (GIST) in advanced stages. This drug is now the standard treatment for GIST. In 2006, a second targeted therapy, sunitinib, was approved for the treatment of GIST, for patients whose GIST is not treatable with imatinib. More recently, imatinib has been approved for use in GIST patients after initial surgery, to try to prevent a recurrence. In addition, imatinib is approved for the treatment of patients with advanced stage of a rare type of sarcoma called dermatofibrosarcoma protuberans. New targeted drugs are being tested in clinical trials. Read more about targeted treatments.

Advanced and recurrent sarcoma

An advanced sarcoma is one that has spread to other sites at the time of diagnosis or recurred after initial treatment. The recurrence may be in the tissue where the sarcoma first appeared (local recurrence) or in another place (regional or distant recurrence).

About one-third of patients treated for soft tissue sarcoma of the arm or leg have a recurrence of the tumor, most likely in the lungs. More than half of those treated for sarcoma of the abdomen or trunk will have some type of recurrence, which can be local, regional, and/or distant. Treatment of the recurrence will depend on the location and type of recurrence and on the method of previous treatment. An isolated local recurrence is usually treated with additional surgery, if possible, plus radiation therapy. Treatment of sarcoma that has spread to other organs or lymph nodes may include surgery alone, surgery plus radiation therapy, surgery plus chemotherapy, chemotherapy alone and also, in the rare cases when the tumor is not growing, a “watch and wait” policy, which is also called active surveillance. Chemotherapy combinations are often used for advanced soft tissue sarcoma. In rare instances, generally in patients with a very large tumor involving the major nerves and blood vessels of the arm or leg, amputation is necessary to control the tumor. This can also be necessary if the tumor grows back in the arm or leg after prior surgery, radiation therapy, and/or chemotherapy.

People who have been treated for sarcoma should have regular follow-up examinations to watch for recurrence, based on a schedule set up by their oncologist (see After Treatment).