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Home > Health Library > Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI]
This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
Soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body.
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues of the body include the following:
Soft tissue sarcomas can form almost anywhere in the body, including the head, neck, and trunk, but are most common in the arms, legs, abdomen, and retroperitoneum.Soft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints.
There are many types of soft tissue sarcoma. The cells of each type of sarcoma look different under a microscope, based on the type of soft tissue in which the cancer began.
For more information about soft tissue sarcomas, see the following:
Having certain inherited disorders can increase the risk of soft tissue sarcoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for soft tissue sarcoma include the following inherited disorders:
Other risk factors for soft tissue sarcoma include the following:
A sign of soft tissue sarcoma is a lump or swelling in soft tissue of the body.
A sarcoma may appear as a painless lump under the skin, often on an arm or a leg. Sarcomas that begin in the abdomen may not cause signs or symptoms until they get very big. As the sarcoma grows and presses on nearby organs, nerves, muscles, or blood vessels, signs and symptoms may include:
Other conditions may cause the same signs and symptoms. Check with your doctor if you have any of these problems.
Soft tissue sarcoma is diagnosed with a biopsy.
If your doctor thinks you may have a soft tissue sarcoma, a biopsy will be done. The type of biopsy will be based on the size of the tumor and where it is in the body. These types of biopsies may be used:
Careful planning of the biopsy should involve the surgeon, a radiation oncologist, and an interventional radiologist who uses medical imaging to guide diagnosis. Samples will be taken from the primary tumor, lymph nodes, and other suspicious areas. A pathologist views the tissue under a microscope to look for cancer cells and to find out the grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade tumors usually grow and spread more quickly than low-grade tumors.
Because soft tissue sarcoma can be hard to diagnose, the tissue samples should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma.
The following tests may be done on the tissue that was removed:
Certain factors affect treatment options and prognosis (chance of recovery).
The treatment options and prognosis depend on the following:
Small, low-grade tumors, especially in the trunk, arms, or legs, are frequently treated with surgery alone. High-grade sarcomas are more difficult to treat and more likely to spread.
After soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread within the soft tissue or to other parts of the body.
The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. Staging of soft tissue sarcoma is also based on the grade and size of the tumor, and whether it has spread to the lymph nodes or other parts of the body. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.
The following tests and procedures may be used in the staging process:
The results of these tests are viewed together with the results of the tumor biopsy to find out the stage of the soft tissue sarcoma before treatment is given. Sometimes chemotherapy or radiation therapy is given as the initial treatment and afterwards the soft tissue sarcoma is staged again.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are actually soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer.
The grade of the tumor is also used to describe the cancer and plan treatment.
The grade of the tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Low grade, mid grade, and high grade are used to describe soft tissue sarcoma:
For soft tissue sarcoma of the trunk, arms, and legs, the following stages are used:
Stage I soft tissue sarcoma of the trunk, arms, and legs is divided into stages IA and IB:
Tumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).
In stage II soft tissue sarcoma of the trunk, arms, and legs, the tumor is 5 centimeters or smaller and is mid grade or high grade.
Stage III soft tissue sarcoma of the trunk, arms, and legs is divided into stages IIIA and IIIB:
In stage IV soft tissue sarcoma of the trunk, arms, and legs, one of the following is found:
For soft tissue sarcoma of the retroperitoneum, the following stages are used:
Stage I soft tissue sarcoma of the retroperitoneum is divided into stages IA and IB:
In stage II soft tissue sarcoma of the retroperitoneum, the tumor is 5 centimeters or smaller and is mid grade or high grade.
Stage III soft tissue sarcoma of the retroperitoneum is divided into stages IIIA and IIIB:
In stage IV soft tissue sarcoma of the retroperitoneum, the tumor is any size, any grade, and may have spread to nearby lymph nodes. Cancer has spread to other parts of the body, such as the lung.
There is no standard staging system for soft tissue sarcoma of the head, neck, chest, or abdomen.
Soft tissue sarcoma can recur (come back) after it has been treated.
The cancer may come back in the same soft tissue or in other parts of the body.
There are different types of treatment for patients with soft tissue sarcoma.
Different types of treatments are available for patients with soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Five types of standard treatment are used:
Surgery is the most common treatment for soft tissue sarcoma. It may be the only treatment needed for small, low-grade tumors, especially in the trunk, arms, or legs. The following surgical procedures may be used:
Radiation therapy or chemotherapy may be given before or after surgery to remove the tumor. When given before surgery, radiation therapy or chemotherapy will make the tumor smaller and reduce the amount of tissue that needs to be removed during surgery. Treatment given before surgery is called neoadjuvant therapy. When given after surgery to remove all of the tumor that can be seen, radiation therapy or chemotherapy will kill any remaining cancer cells. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy and internal radiation therapy may be used to treat soft tissue sarcoma.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).
For more information, see Drugs Approved for Soft Tissue Sarcoma.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapy may cause less harm to normal cells than chemotherapy or radiation therapy do. There are different types of targeted therapy. These include:
Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy.
Immune checkpoint inhibitor therapy is a type of immunotherapy. Some types of immune system cells, such as T cells, and some cancer cells have certain proteins, called checkpoint proteins, on their surface that keep immune responses in check. These checkpoints help keep immune responses from being too strong and sometimes can keep T cells from killing cancer cells. When these checkpoints are blocked, T cells can kill cancer cells better.
Types of immune checkpoint inhibitor therapy include the following:
Ipilimumab is a type of CTLA-4 inhibitor that is being studied to treat soft tissue sarcoma.
Pembrolizumab and nivolumab are PD-1 inhibitors that are used to treat progressive and recurrent soft tissue sarcoma.
Immune checkpoint inhibitor. Checkpoint proteins, such as PD-L1 on tumor cells and PD-1 on T cells, help keep immune responses in check. The binding of PD-L1 to PD-1 keeps T cells from killing tumor cells in the body (left panel). Blocking the binding of PD-L1 to PD-1 with an immune checkpoint inhibitor (anti-PD-L1 or anti-PD-1) allows the T cells to kill tumor cells (right panel).
Treatment for soft tissue sarcoma may cause side effects.
For information about side effects caused by treatment for cancer, see our Side Effects page.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
For information about the treatments listed below, see Treatment Option Overview.
Treatment of stage I soft tissue sarcoma may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of stage II soft tissue sarcoma and stage III soft tissue sarcoma that has not spread to lymph nodes may include the following:
Treatment of stage III soft tissue sarcoma that has spread to lymph nodes (advanced) may include the following:
Treatment of stage IV soft tissue sarcoma may include the following:
Treatment of recurrent soft tissue sarcoma may include the following:
For more information from the National Cancer Institute about soft tissue sarcomas, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
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Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of adult soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
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Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
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The best way to cite this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389216]
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Last Revised: 2022-04-07
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