ON THIS PAGE: You will learn about the different types of treatments doctors use for people with these types of sarcoma. Use the menu to see other pages.
This section explains the types of treatments that are the standard of care for this type of sarcoma. “Standard of care” means the best treatments known. When making treatment plan decisions, you are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option to consider for treatment and care for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.
In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Cancer care teams include a variety of other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.
Treatment options and recommendations depend on several factors, including the type and stage of the sarcoma, the location of the tumor, possible side effects, and the patient’s preferences and overall health.
Because there are so many different types of sarcoma, and treatment plans should be individualized, it is not possible to describe the best treatments for each of the rare sarcomas in this section. However, descriptions of the common types of treatments used for sarcomas of specific organs are listed below. It is strongly recommended that people who are diagnosed with a rare type of sarcoma talk with a doctor at a sarcoma expert center. There may be new drugs or other treatments in clinical trials that may be the best option for you. Talk with your doctor about finding a specialist center. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care.
Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of talks are called “shared decision making.” Shared decision making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision making is particularly important for sarcomas because there are different treatment options. Learn more about making treatment decisions.
Surgery is the removal of the tumor and some surrounding healthy tissue, called the margin, during an operation. A surgical oncologist is a doctor who specializes in treating cancer using surgery.
Surgery is the most common treatment for most sarcomas that develop in a specific organ. If the tumor is in an arm or leg, surgical techniques can often be used to avoid removing the limb. However, because the surgeon may need to take a wide margin of tissue to make sure no cancer remains, occasionally there may be a need to remove part or all of a limb, called amputation. If amputation is needed, rehabilitation that includes physical therapy can help a patient have the most physical function possible. Rehabilitation can also help a person cope with the social and emotional effects of losing a limb.
Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of cancer surgery.
Some types of sarcoma cannot be removed using surgery. For example, epithelioid hemangioendothelioma of the liver usually affects many parts of the liver at once and other parts of the body. As a result, surgery, even liver transplantation, cannot completely eliminate the cancer. This is similar to the situation for 80% of people with cardiac sarcoma. By the time the cardiac tumor causes symptoms, it has already spread and cannot be completely removed with surgery. In these situations, radiation therapy or chemotherapy will typically be recommended instead (see below).
Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.
Radiation therapy may be used before surgery to shrink the size of the tumor or after surgery to destroy any remaining cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.
Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.
Learn more about the basics of radiation therapy.
Therapies using medication
Systemic therapy is the use of medication to destroy cancer cells. This type of medication is given through the bloodstream to reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.
Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).
The types of systemic therapies used for sarcoma include:
Each of these types of therapies is discussed below in more detail. A person may receive 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.
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. It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications. Learn more about your prescriptions by using searchable drug databases.
Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells.
A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. The type of chemotherapy your doctor recommends will depend on multiple factors, including the type of sarcoma and whether you have other medical problems. Commonly used chemotherapies include but are not limited to doxorubicin (available as a generic drug), ifosfamide (Ifex), gemcitabine (Gemzar), docetaxel (Taxotere), paclitaxel (available as a generic drug), trabectedin (Yondelis), eribulin (Halaven), and dacarbazine (DTIC-Dome).
The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.
Learn more about the basics of chemotherapy.
Kinase inhibitors are a type of systemic treatment called targeted therapy that blocks the function of a protein found in sarcoma cells. This type of drug blocks the growth and spread of cancer cells and limits damage to healthy cells.
Pazopanib (Votrient) is an approved targeted therapy for sarcomas that are not stopped by other treatments. Pazopanib is not used for liposarcoma or GIST. The drug starves sarcoma cells of a blood supply, which helps slow tumor growth and sometimes shrinks sarcomas. The most common side effects of pazopanib are diarrhea, fatigue, loss of coloration in the hair and skin, and high blood pressure. Talk with your doctor about possible side effects for a specific medication and how they can be managed. Learn more about the basics of targeted treatments.
A small percentage of sarcomas, less than 1%, have a mutation in the neurotrophic receptor tyrosine kinase (NTRK) gene. Larotrectinib (Vitrakvi) is an NTRK inhibitor that is now approved for any cancer that has a specific mutation in the NTRK gene. The most common side effects include fatigue, nausea, dizziness, vomiting, increased liver enzymes, cough, constipation, and diarrhea.
Clinical trials are taking place to find out more about new treatments for rare sarcomas unique to specific body parts. See the Latest Research section for more information.
Immunotherapy (updated 09/2021)
Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function.
Immunotherapy is generally not approved for the treatment of sarcomas because they have not been tested well enough for this specific type of cancer. Many recent approved immunotherapy treatments for other types of cancer involve “immune checkpoint inhibitors.” These drugs are given to take the brakes off the body’s natural immune response against the cancer in the body. The current methods of immunotherapy do have problems because these drugs also activate immune responses against normal body parts, a process called autoimmunity. Some of these drugs are approved to treat other cancers. However, if testing on your tumor shows that it has specific problems with repairing DNA damage, called microsatellite instability-high (MSI-H) or mismatch repair deficiency (dMMR; this occurs in less than 1% of sarcomas), then a checkpoint inhibitor called pembrolizumab (Keytruda) or dostarlimab (Jemperli) may be used.
In uncommon situations, white blood cells can be trained to fight cancer. If a specific target can be identified in your cancer, specially engineered T cells from your own body are used to attack the cancer cells. This kind of immunotherapy is called chimeric antigen receptor (CAR) T-cell therapy. At present, these cellular treatments are given only in clinical trials for people with specific types of sarcoma.
Different types of immunotherapy can cause different side effects. Common side effects include skin reactions, flu-like symptoms, diarrhea, and weight changes. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.
Organ transplantation involves replacing an organ affected by sarcoma with a healthy organ from a donor. Organ transplant is very rarely used to treat sarcomas; however, examples include heart transplantation as a treatment for cardiac sarcoma and liver transplantation to treat an epithelioid hemangioendothelioma that is only growing in the liver.
For a transplant to be successful, the patient will have to take immunosuppressive medication to help the patient’s body accept the new organ. As a result of taking this medication, the patient could develop a new cancer or the sarcoma might come back. In addition, people may have to wait a long time for a donor organ to become available. Therefore, patients and their doctors should carefully consider and talk about whether this treatment option is right for them.
Physical, emotional, and social effects of cancer
Sarcoma and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the cancer.
Palliative care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative care along with treatment for the sarcoma often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.
Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments similar to those meant to get rid of the cancer, such as chemotherapy, surgery, or radiation therapy.
Before treatment begins, talk with your doctor about the goals of each treatment in the treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options.
During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.
If sarcoma spreads to another part in the body from where it started, doctors call it metastatic sarcoma. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.
Your treatment plan may include a combination of systemic therapy using medication, radiation therapy, and surgery. Palliative care will also be important to help relieve symptoms and side effects.
For many people, a diagnosis of metastatic sarcoma is very stressful and difficult. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.
Remission and the chance of recurrence
A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.
A remission can be temporary or permanent. While many remissions are permanent, it is important to talk with your doctor about the possibility of the sarcoma returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the sarcoma does return. Learn more about coping with the fear of recurrence.
If the sarcoma returns after the original treatment, it is called recurrent sarcoma. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).
When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as surgery, chemotherapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat this type of recurrent sarcoma. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.
People with recurrent sarcoma sometimes experience emotions such as disbelief or fear. You are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.
If treatment does not work
Recovery from sarcoma is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.
This diagnosis is stressful, and for some people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.
People who have advanced cancer and who are expected to live less than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.
After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.
The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with cancer. Use the menu to choose a different section to read in this guide.