ON THIS PAGE: You will find information about the estimated number of people who will be diagnosed with soft-tissue sarcoma each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors, and no 2 people with cancer are the same. Use the menu to see other pages.

Every person is different, with different factors influencing their risk of being diagnosed with this cancer and the chance of recovery after a diagnosis. It is important to talk with your doctor about any questions you have around the general statistics provided below and what they may mean for you individually. The original sources for these statistics are provided at the bottom of this page.

How many people are newly diagnosed with soft-tissue sarcoma?

In 2023, an estimated 13,400 people (7,400 men and boys and 6,000 women and girls) in the United States will be diagnosed with soft-tissue sarcoma.

It is estimated that 5,140 deaths (2,720 men and boys and 2,420 women and girls) from this disease will occur in the United States in 2023.

Sarcomas are described as being localized, locally advanced, or metastatic when they are first found.

• Localized means the tumor is only in 1 area of the body.

• Locally advanced means the tumor involves or attaches to nearby tissues or organs. This often means it is not possible to remove the tumor because of its location, size, or the organs it involves.

• Metastatic means the sarcoma has spread to parts of the body far away from where the sarcoma started.

This page includes information on how often sarcomas show up as localized, locally advanced, or metastatic. In addition, there is information on survival rates for each situation. This information applies for sarcomas in general and may not apply to a specific type of sarcoma. Talk with your health care team for more information regarding your specific diagnosis.

What is the survival rate for soft-tissue sarcoma?

There are different types of statistics that can help doctors evaluate a person’s chance of recovery from sarcoma. These are called survival statistics. A specific type of survival statistic is called the relative survival rate. It is often used to predict how having cancer may affect life expectancy. Relative survival rate looks at how likely people with sarcoma are to survive for a certain amount of time after their initial diagnosis or start of treatment compared to the expected survival of similar people without this cancer.

Example: Here is an example to help explain what a relative survival rate means. Please note this is only an example and not specific to this type of cancer. Let’s assume that the 5-year relative survival rate for a specific type of cancer is 90%. “Percent” means how many out of 100. Imagine there are 1,000 people without cancer, and based on their age and other characteristics, you expect 900 of the 1,000 to be alive in 5 years. Also imagine there are another 1,000 people similar in age and other characteristics as the first 1,000, but they all have the specific type of cancer that has a 5-year survival rate of 90%. This means it is expected that 810 of the people with the specific cancer (90% of 900) will be alive in 5 years.

It is important to remember that statistics on the survival rates for people with sarcoma are only an estimate. They cannot tell an individual person if cancer will or will not shorten their life. Instead, these statistics describe trends in groups of people previously diagnosed with the same disease, including specific stages of the disease.

The 5-year relative survival rate for sarcoma is over 65%.

The survival rates for sarcoma vary based on several factors. These include the stage and grade of cancer, a person’s age and general health, and how well the treatment plan works. Other factors that can affect outcomes include the type of sarcoma and where it is located.

About 59% of sarcomas are found as a localized sarcoma. The 5-year relative survival rate for people with localized sarcoma is over 81%.

About 19% of sarcomas are found in a locally advanced stage. The 5-year relative survival rate for people with locally advanced sarcoma is 58%.

About 16% of sarcomas are found in a metastatic stage. The 5-year relative survival rate for people with metastatic sarcoma is 17%.

Experts measure relative survival rate statistics for sarcoma every 5 years. This means the estimate may not reflect the results of advancements in how sarcoma is diagnosed or treated from the last 5 years. Talk with your doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society website and the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program. (All sources accessed March 2023.)

The next section in this guide is Medical Illustrations. It offers a drawing of body parts often affected by soft-tissue sarcoma. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find out more about the factors that increase the chance of developing sarcoma. Use the menu to see other pages.

What are the risk factors for sarcoma?

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. Knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

Most sarcomas do not have known causes. The following factors may raise a person’s risk of developing sarcoma:

• Previous radiation therapy. People who were treated with radiation therapy for a previous cancer, usually more than 5 years ago, have a slightly increased risk of later developing sarcoma in the area where the radiation was directed.

• Genetics. People with certain inherited conditions have a higher risk of sarcoma. These diseases include neurofibromatosis type 1, also known as von Recklinghausen’s disease; familial adenomatous polyposis, also known as Gardner syndrome; Werner syndrome; tuberous sclerosis complex; nevoid basal cell carcinoma syndrome; Li-Fraumeni syndrome; and retinoblastoma.

• Immune system problems. People with problems with their immune system have a higher risk of several types of cancer, whether from infections such as human immunodeficiency virus (HIV), cancers such as chronic lymphocytic leukemia, or autoimmune conditions such as lupus or psoriasis.

• Lymphedema. People who have surgery or radiation therapy for other reasons may have swelling of an arm, leg, or other part of the body as a side effect of the treatment. This is called lymphedema. For example, lymphedema can happen after breast surgery when lymph nodes are removed from the armpit area. People can also be born with lymphedema. Sarcomas, such as angiosarcoma, occasionally form in areas where lymphedema had developed.

• Chemicals. Workplace exposure to vinyl chloride monomer, which is used in making some types of plastics, Agent Orange, or dioxin may increase the risk of sarcoma. However, most sarcomas are not known to be associated with specific environmental hazards.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems sarcoma can cause. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, cancer. They also do tests to learn if cancer has spread to another part of the body from where it started. If cancer has spread, it is called metastasis. Doctors may also do tests to learn which treatments may work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Although biopsies have a small chance of not giving a definite answer, they are very important to allow your doctor to make a clear diagnosis and develop a team-based treatment plan.

How sarcoma is diagnosed

There are different tests used for diagnosing sarcoma. Not all tests described here will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

• The type of cancer suspected

• Your signs and symptoms

• Your age and general health

• The results of earlier medical tests

There are no standard screening tests for sarcoma. A doctor should examine any unusual or new lumps or bumps that are growing or, in adults, are larger than 2 inches (5 centimeters; or smaller in young children) to make sure it is not cancer. If a sarcoma is suspected, it is very important to talk with a doctor who has experience with this type of cancer.

A diagnosis of sarcoma is made by a combination of clinical examination by a doctor and imaging tests. It is confirmed by the results of a biopsy. In addition to a physical examination, some of the tests described below may be used to diagnose sarcoma.

Imaging tests

Imaging tests show pictures of the inside of the body. Both benign and cancerous tumors can show up on imaging tests, such as an x-ray. A radiologist, a medical doctor who performs and interprets imaging tests to diagnose disease, will use the way the tumor looks on the test to help determine whether it may be benign or cancerous. However, a biopsy is almost always needed.

• X-ray. An x-ray creates a picture of the structures inside of the body using a small amount of radiation. X-rays are particularly useful for bone sarcomas but less valuable for soft-tissue sarcomas.

• Ultrasound. An ultrasound creates a picture of the structures inside the body using sound waves. It may be used to look at lumps under the skin or other organs in the body. A vaginal ultrasound may be used to diagnose sarcoma of the uterus.

• Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size or see if the cancer has spread somewhere else. Sometimes, a special dye called contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein and/or be a pill or liquid the patient must drink.

• Magnetic resonance imaging (MRI). An MRI produces detailed images of the inside of the body using magnetic fields, not x-rays. MRI can be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein. MRI is often used to determine whether a sarcoma can be removed with surgery.

• Positron emission tomography (PET) or PET-CT scan. A PET scan creates pictures of organs and tissues inside the body. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. However, the amount of radiation in the substance is too low to be harmful. A scanner then detects this substance to produce images of the inside of the body. This technique can be used to look at both the tumor’s structure and how much energy is used by the tumor and normal tissues. This information can be helpful in planning treatment and evaluating how well treatment is working, but it is usually not performed in all cases of known or suspected soft-tissue sarcoma.

Tests for sarcomas in specific organs

• Mammography. A mammogram is a type of x-ray that looks for any abnormalities or tumors in the breast. This imaging test may be used with MRI scans, CT scans, and a biopsy to diagnose a sarcoma of the breast.

• Bone scan. A bone scan looks at the inside of the bones using a radioactive tracer. The amount of radiation in the tracer is too low to be harmful. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears lighter to the camera, and areas of injury, such as those caused by cancer, stand out on the image.

• Heart evaluation. A heart evaluation, including an electrocardiogram (EKG or ECG) and an echocardiogram (ECHO), will look for structural abnormalities in the organ and motion of the walls of the heart. This evaluation is used to diagnose cardiac sarcoma.

Biopsy and tissue tests

Imaging tests may suggest the diagnosis of sarcoma, but a biopsy will be needed to confirm the diagnosis and to find out the type of sarcoma. Because a biopsy that is not properly done can make a surgery more difficult, whenever a sarcoma is suspected, it is very important for a patient to see a sarcoma specialist before any surgery or biopsy is done.

Biopsy

A biopsy is the only way to make a reliable diagnosis, even if other tests can suggest that cancer is present. During biopsy, a small amount of tissue is removed for examination under a microscope. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

Because soft-tissue sarcomas are rare, an expert pathologist should review the tissue sample to properly diagnose a sarcoma. Sometimes properly diagnosing a sarcoma requires special tests on the tumor tissue, and it is best if a specialist who sees this type of cancer regularly does this.

There are different types of biopsies.

• For a needle biopsy, a doctor removes a small sample of tissue from the tumor with a needle-like instrument—usually a core needle biopsy. This may be performed with the help of ultrasound, CT scan, or MRI to precisely guide the needle into the tumor.

• In an incisional biopsy, a surgeon cuts into the tumor and removes a sample of tissue.

• In an excisional biopsy, the surgeon removes the entire tumor. Excisional biopsies are generally not recommended for sarcomas because the risk of local recurrence is very high and second surgeries are often needed to completely remove the tumor. A recurrence is when cancer comes back after treatment.

If a biopsy is not optimally performed, it can make surgery more difficult. That is one of the many reasons why a patient should be seen in a sarcoma specialty center before the biopsy is performed, so that the treating surgeon can identify the location for the biopsy. It is also very important to have an expert pathologist review the sample of tissue to appropriately diagnose a sarcoma.

Tissue testing of the tumor

Your doctor or the pathologist looking at the sarcoma may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide what the treatment should be, because each sarcoma can be as different from one another as breast cancer is different from colon cancer.

After diagnostic tests are done, your doctor will review the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer. This is called staging and grading.

The next section in this guide is Stages and Grades. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with sarcoma. Use the menu to see other pages.

This section explains the types of treatments, also known as therapies, that are the standard of care for sarcoma. “Standard of care” means the best treatments known. When making treatment plan decisions, you are encouraged to discuss with your doctor whether clinical trials are an option. A clinical trial is a research study that tests a new approach to treatment. Doctors learn through clinical trials whether a 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 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.

How sarcoma is treated

Sarcoma is rare, and research shows that patients have better outcomes if they are treated at a medical center with experience treating sarcomas. These are called "sarcoma specialty centers."

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, stage, and grade of sarcoma, possible side effects, and the patient’s preferences and overall health. 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.

The common types of treatments used for sarcomas are described below. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. 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.

• Surgery

• Radiation therapy

• Chemotherapy

• Targeted therapy

• Immunotherapy

• Organ transplantation

• Physical, emotional, and social effects of cancer

• Treatment by stage of sarcoma

• Remission and the chance of recurrence

• If treatment does not work

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. Before surgery, it is important to have a biopsy and appropriate imaging scans to confirm the diagnosis (see Diagnosis). After a biopsy, surgery is typically an important part of the treatment plan if the tumor is localized (located in only 1 area). Surgical oncologists and orthopedic oncologists are doctors who specialize in treating sarcomas using surgery.

The surgeon's goal is to remove the tumor and enough normal tissue surrounding it to obtain a clean margin around the tumor. A “clean margin” means there are no tumor cells visible at the borders of the surgical specimen. This is currently the best method available to ensure that there are no tumor cells left in the area from which the tumor was removed. Small low-grade sarcomas can usually be effectively removed by surgery alone. Those that are high grade and larger than 2 inches (5 cm) are often 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. They also may be used during and after surgery to destroy any remaining cancer cells.

Rarely, for patients with a very large tumor involving the major nerves and blood vessels of the arm or leg, surgical removal of the limb is required to control the tumor. This is called amputation. This surgery can also be necessary if the tumor grows back in the arm or leg after surgery, radiation therapy, and/or chemotherapy have been completed. It’s important to remember that the operation that results in the most useful and strongest limb may be different from the one that gives the most normal appearance. If amputation is needed, rehabilitation, including physical therapy, can help maximize physical function. Rehabilitation can also help a person cope with the social and emotional effects of losing a limb. People who have had an amputation can often wear a prosthesis, which is an artificial body part, depending on the type of amputation.

If the sarcoma is found at an early stage and has not spread from where it started, surgical treatment is often very effective and many people are cured. However, if the sarcoma has spread to other parts of the body, treatment can usually control the tumor but not cure it.

Some sarcomas cannot be removed using surgery. Some sarcomas cannot be reached surgically or may involve critical structures that cannot be removed. 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).

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have, including the recovery period. Learn more about the basics of cancer surgery.

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Radiation therapy

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. Since sarcomas are rare, it is very important to talk with a radiation oncologist who has experience treating sarcomas.

A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.

Radiation therapy may be done before surgery to shrink the tumor so that it may be 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 important structures in the arm or leg if the sarcoma is located in any of those places.

Radiation therapy can damage normal cells, but because it is focused around the tumor, side effects are usually limited to those areas.

External-beam radiation therapy

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body.

The way external-beam radiation is used has changed over the past 20 years. It is now possible to give many small beams of radiation that turn on and off as the radiation machine rotates around the body. This is called intensity modulated radiation therapy (IMRT), and this approach is commonly used for sarcomas. IMRT focuses more radiation on the tumor site and less on the normal tissues. As a result, there are fewer side effects than there were in the past. Similarly, stereotactic body radiation treatment (SBRT) is another type of advanced radiation therapy that is used with image guidance to treat a small tumor (often smaller than 5 cm).

Proton beam radiation therapy

Proton therapy is a type of external-beam radiation treatment that uses protons rather than x-rays. Like x-rays, protons can destroy cancer cells. It is most commonly used in parts of the body close to critical structures, for example, near the spinal cord or at the base of the brain. Learn more about proton therapy. In addition, radiation treatment using heavier charged particles, known as carbon-ion radiation therapy, is being studied in Japan, Germany, and China for the treatment of sarcomas.

Intraoperative radiation therapy

In some hospitals, part of the planned radiation therapy can be given during surgery. This approach can decrease the need to expose healthy tissue to radiation from external-beam radiation or brachytherapy.

Brachytherapy

Brachytherapy is the insertion of radiation seeds through thin tubes called catheters directly into the affected area of the body. Brachytherapy usually requires specialized skills and special training. It is only used in certain hospitals and only in special situations to treat sarcoma.

Side effects of radiation therapy

Side effects from radiation therapy depend on what part of the body receives radiation. They may include fatigue, mild skin reactions, upset stomach, and loose bowel movements.

In the short term, radiation can cause injury to the skin that looks like a sunburn. This is usually treated with creams that keep the skin soft and help relieve discomfort. Radiation therapy can also affect wound healing. Most side effects go away soon after treatment is finished.

In the long term, radiation can cause scarring that affects the function of an arm or a leg. Radiation therapy can damage the bowels, liver, kidneys, or other internal organs when it is used to treat soft-tissue sarcoma of the retroperitoneum, abdomen, or pelvis.

In rare cases, radiation can cause another sarcoma or other cancer. In the unlikely event that this happens, it can be 7 to 20 years after radiation for a second cancer to develop. Each person is encouraged to talk with their doctor about the possible risks and benefits of the radiation therapy recommended for them. Learn more about radiation therapy.

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Therapies using medication

The treatment plan may include medications to destroy cancer cells. Medication may be given through the bloodstream to reach cancer cells throughout the body. When a drug is given this way, it is called systemic therapy.

This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Medications are often given through an intravenous (IV) tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). If you are given oral medications, be sure to ask your health care team about how to safely store and handle them.

The types of medications used for sarcomas include:

• Chemotherapy

• Targeted therapy

• Immunotherapy

Each of these types of therapies is discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications 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, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.

Chemotherapy

Chemotherapy is medication that keeps cancer cells from growing, dividing, and making more cells. Cancer cells grow and divide faster than normal cells. However, normal cells also grow and divide, so the side effects of chemotherapy are due to the treatment’s effects on normal cells that are growing and dividing.

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. Chemotherapy for sarcoma can usually be given as an outpatient treatment.

Different drugs are used to treat different types and subtypes of sarcoma. Some types of chemotherapy that might be used alone or in combination for sarcoma include:

• Doxorubicin (available as a generic drug)

• Epirubicin (Ellence)

• Ifosfamide (Ifex)

• Gemcitabine (Gemzar)

• Docetaxel (Taxotere)

• Paclitaxel (available as a generic drug)

• Trabectedin (Yondelis)

• Eribulin (Halaven)

• Dacarbazine (available as a generic drug)

This is a condensed list of some common chemotherapies for sarcoma, since there are over 70 types of soft-tissue sarcoma. There are several other chemotherapies that may be used to treat different types of sarcomas. In some cases, a specific drug or drugs are used only for a particular type of sarcoma.

Chemotherapy is often used when a sarcoma has already spread. It may be given alone or in combination with surgery, radiation therapy, or both.

For example, certain types of sarcomas may be treated with chemotherapy before surgery to make the tumor easier to remove. Chemotherapy given before surgery may be called by different names, including preoperative chemotherapy, neoadjuvant chemotherapy, or induction chemotherapy.

If a patient has not received chemotherapy before surgery, chemotherapy may be given to destroy any microscopic tumor cells that remain after a patient has recovered from surgery. Chemotherapy given after surgery is called adjuvant chemotherapy or postoperative chemotherapy.

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. In rare cases, there are long-term problems that affect the heart or kidneys or cause second cancers.

Learn more about the basics of chemotherapy.

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Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival, usually by blocking the action of proteins in cells called kinases. This type of treatment blocks the growth and spread of cancer cells and limits damage to healthy cells.

Not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.

Targeted therapies for soft-tissue sarcoma include:

Imatinib (Gleevec). Imatinib is a type of targeted therapy called a tyrosine kinase inhibitor, also called a TKI. It is the standard first-line treatment for GIST worldwide. Imatinib is also approved for the treatment of people with advanced-stage dermatofibrosarcoma protuberans (DFSP). Two other targeted drugs, sunitinib (Sutent) and regorafenib (Stivarga), are approved for the treatment of GIST when imatinib does not work.

Pazopanib (Votrient). Pazopanib is a type of targeted therapy called a multitargeted tyrosine kinase inhibitor. It is approved to treat kidney cancer as well as several types of soft-tissue sarcoma.

Tazemetostat (Tazverik). Tazemetostat is a targeted therapy that targets EZH2. The U.S. Food and Drug Administration (FDA) approved it for the treatment of people 16 and older with epithelioid sarcoma that cannot be removed with surgery. Epithelioid sarcoma is a rare type of sarcoma that often starts in the fingers, hands, arms, or feet of young adults.

Pexidartinib (Turalio). Pexidartinib is a colony-stimulating factor-1 (CSF-1) inhibitor that the FDA has approved to treat certain people with tenosynovial giant cell tumors (TGCT), sometimes called pigmented villonodular synovitis or giant cell tumor of tendon sheath. These are rare tumors that affect the tendons and joints of younger adults. In a recent clinical trial, of the 61 people who took pexidartinib, the drug worked in 39.3% of them and improved their pain, range of motion, and physical function. Pexidartinib can be used to treat people with significant symptoms from TGCT for whom surgery is not considered a good option. Taking pexidartinib did cause serious or potentially fatal side effects that affected the liver in some patients. Therefore, it can only be prescribed by a certified specialist, and patients must enroll in a patient registry.

Sirolimus protein-bound particles (Fyarro). Sirolimus is a kinase inhibitor that targets the mammalian target of rapamycin (mTOR). This medication may be given as an infusion to treat perivascular epithelioid cell tumor (PEComa) that is locally advanced and cannot be removed with surgery, as well as metastatic malignant PEComa. PEComa is a type of rare tumor that forms in the soft tissues of the stomach, intestines, lungs, female reproductive organs, and genitourinary organs. Most PEComas are benign. They often occur in children with an inherited condition called tuberous sclerosis.

Tumor-agnostic treatments. A small percentage of sarcomas, less than 1%, have a mutation in the neurotrophic receptor tyrosine kinase (NTRK) gene. Larotrectinib (Vitrakvi) and entrectinib (Rozlytrek) are NTRK inhibitors that are approved for any cancer that has a specific gene fusion in the NTRK gene.

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.

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Immunotherapy (updated 01/2023)

Immunotherapy uses the body's natural defenses to fight cancer by improving your immune system’s ability to attack cancer cells.

Immunotherapy is generally not approved for the treatment of sarcomas because they have not yet shown significant benefit in most sarcomas. Many recently approved immunotherapy treatments for other types of cancers 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 immunotherapy treatments can cause problems because these drugs also activate immune responses against normal body parts, leading to side effects from an overactive immune system.

Atezolizumab (Tecentriq). Atezolizumab is a type of immune checkpoint inhibitor. It is approved for alveolar soft part sarcoma.

Tumor-agnostic treatments. In a small percentage of sarcomas (less than 1%), testing on a tumor may show that it has high tumor mutational burden or specific problems with repairing DNA damage, called microsatellite instability-high (MSI-H) or mismatch repair deficiency (dMMR). If these problems are present, then a checkpoint inhibitor called pembrolizumab (Keytruda) or dostarlimab (Jemperli) may be used.

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.

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Organ transplantation

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, there are examples of heart transplantation used as a treatment for cardiac sarcoma and liver transplantation used 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 donated organ. As a result of taking this medication, a new cancer may develop 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.

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Physical, emotional, and social effects of cancer

Cancer 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 cancer 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 recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

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.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.

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Treatment by stage of sarcoma

Different treatments may be recommended for each stage of sarcoma. Below are generalized treatment options by stage. For more detailed descriptions, see “How sarcoma is treated,” above. Your doctor will work with you to develop a specific treatment plan for you based on the cancer’s stage and other factors. Clinical trials may also be a treatment option for each stage.

When considering the treatment plan, doctors will often divide sarcomas into 2 categories: curable and treatable. Curable sarcomas can be completely removed from the body, with the goal of preventing it from coming back. Treatable sarcomas cannot be totally removed from the body but can be controlled for some time with treatments. In many cases, stage I to stage III sarcoma is curable and stage IV, or metastatic, sarcoma is treatable.

Stage I sarcoma

At this early stage, sarcoma can often be completely removed with surgery. Treatment with radiation therapy before or after surgery is sometimes recommended.

Stage II sarcoma

Stage II sarcoma is often high grade and can grow and spread quickly. Treatments at this stage include surgery plus radiation therapy. If the tumor is hard to reach, radiation therapy may be used first to shrink the tumor. This is called neoadjuvant treatment. Or, if the tumor can be removed with surgery, radiation therapy may be used afterward to reduce the risk of the cancer coming back. This is called adjuvant treatment. There are risks and benefits to giving radiation therapy before versus after surgery, and the decision to use it must be made based on a person's individual situation.

Stage III sarcoma

Stage III sarcoma is also high grade and larger. Typically treatment will involve a combination of surgery and radiation therapy. Chemotherapy may also be added to the treatment plan. Radiation therapy, chemotherapy, or both may be given before and/or after surgery to shrink tumors and lower the risk of the cancer coming back.

Metastatic (stage IV) sarcoma

If cancer spreads to another part in the body from where it started, doctors call it metastatic cancer. 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 at a sarcoma specialty center before starting treatment, so you are comfortable with your chosen treatment plan.

Your treatment plan may include medications as described above and other palliative treatments to help relieve symptoms and side effects. In addition to providing symptom relief, medical treatments such as chemotherapy may also slow the spread of the cancer. The type of medical treatment that is recommended will depend on many factors, such as the type of sarcoma, which treatments you have received before, and your medical history. Clinical trials involving new drugs or combinations of drugs may also be considered.

Surgery may be used to remove individual metastases, especially if the cancer has spread to a lung, but only a small percentage of people benefit from this. This surgical procedure is called metastatectomy. Radiation therapy can also be used to help relieve symptoms and side effects. If cancer has spread to the liver, localized treatments may be recommended, such as surgery, radiation therapy, and chemotherapy. Occasionally, when the tumor is not growing, a “watch and wait” approach, also called “active surveillance,” may be used. This means that the patient is closely monitored and active treatment begins only if the tumor begins to grow or cause symptoms.

For many people, a diagnosis of metastatic cancer 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 your health care team. It may also be helpful to talk with other patients, such as through a support group or other peer support program.

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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 may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer returns after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence). If the sarcoma was originally in the arm or leg, the recurrence most commonly occurs in the lungs. Patients treated for sarcoma of the abdomen or torso are at risk for local, regional, or distant recurrence.

If a recurrence happens, a new cycle of testing will begin again to learn as much as possible about it. 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, therapies using medication, 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 recurrent sarcoma. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

A local recurrence often can be successfully treated with additional surgery plus radiation therapy, but the risks of side effects from these treatments tends to increase. Treatment for a distant recurrence is most successful when there are a small number of tumors that have spread to the lung that can be completely removed surgically, destroyed with radiofrequency ablation (see below), or destroyed with ablative high-dose radiation therapy (also known as stereotactic body radiotherapy, SBRT, or Gamma Knife radiotherapy):

• Radiofrequency ablation is a technique where a needle is inserted into the tumor to deliver an electrical current that can destroy the cancer. This burns the tumor from the inside out.

• SBRT is the use of pinpointed radiation at very high doses over a few treatments to attack a specific small area of tumor. This is a useful technique because it uses fewer treatments and can be more precise than external-beam radiation therapy.

People with recurrent cancer sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.

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If treatment does not work

Recovery from sarcoma is not always possible. If the sarcoma cannot be cured, it can often still be controlled, at least for a period of time. It is important to understand that patients can live with cancer in their body as long as it does not affect the function of a major organ. Therefore, the goal of treatment is to control the cancer and preserve organ function.

If the sarcoma can no longer be controlled, it is called end-stage or terminal cancer. This diagnosis is stressful, and advanced cancer is difficult to discuss for some people. 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 fewer 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 cope with the loss. Learn more about grief and loss.

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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.

ON THIS PAGE: You will read about the scientific research being done to learn more about sarcomas and how to treat them. Use the menu to see other pages.

Doctors are working to learn more about sarcoma, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the best diagnostic and treatment options for you.

• Immunotherapy. As discussed in Types of Treatment, immunotherapy comes in many forms. Immune checkpoint inhibitors have become the focus of a lot of research. These drugs are targeted monoclonal antibodies (proteins) that turn on immune responses in the body by taking the brakes off the immune system. The molecules that are blocked have names such as CTLA-4, PD-1, PD-L1, OX40, LAG3, and TIM3. This approach has proved helpful in treating many cancers and in research studies about sarcomas.
  
  Chimeric antigen receptor (CAR) T-cell therapy and T-cell receptor therapy use white blood cells from a patient’s blood to destroy cancer cells. The patient’s cells are removed and then changed so they have specific proteins called receptors. These receptors allow those T cells to recognize the cancer cells. The changed T cells are grown in large numbers and returned to the patient’s body. Once in the patient’s body, these T cells seek out and destroy cancer cells. This technique shows the most promise when there is a known target on the sarcoma, such as in synovial sarcoma and myxoid-round cell liposarcoma. Clinical trials are investigating this exciting but complex form of immunotherapy. Learn more about the basics of CAR T-cell therapy.

  Vaccines against specific sarcoma proteins or other molecules are also being studied, often in addition to immune checkpoint inhibitors. This type of vaccine is intended to treat cancer.

• Improved drug delivery. Some chemotherapies are incorporated into fat molecules called liposomes to improve the absorption and distribution of the drug in the patient’s body. Other new ways to get a chemotherapy into a cancer cell are being studied. For example, chemotherapy can be attached to proteins, so the chemotherapy can enter into the cancer cells. In some cases, these proteins are antibodies that target, or bind to, a specific marker on a cancer cell, so the chemotherapy is delivered only to cancer cells and not to normal cells, which can improve effectiveness and decrease side effects. These are called antibody-drug conjugates.

• New drugs. New medications are being developed and tested that may be effective in treating some subtypes of soft-tissue sarcoma. Learn more about the process of drug development and approval. As explained in Types of Treatment, several targeted therapies have been recently approved to treat specific types of sarcoma. This is an active area of research for sarcoma.

• Targeted therapy. As described in Types of Treatment, targeted therapy is a treatment that targets the tumor’s specific genes, proteins, or the tissue environment that contributes to tumor growth and survival. Clinical trials led to the approval of a targeted drug, pazopanib, to treat some sarcomas. Clinical trials of other medications that work in different ways to block tumor growth and survival are also underway.

• Tumor genetics. Researchers are learning that some sarcomas have unique genetic “fingerprints.” Understanding these fingerprints may help doctors make precise diagnoses, determine better treatments, and possibly better predict a patient’s prognosis. A number of cancer centers and companies now offer genetic tests of cancers to determine whether people with a sarcoma might benefit from newer treatments.

• Palliative care/supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current sarcoma treatments to improve comfort and quality of life for patients.

Looking for More About the Latest Research?

If you would like more information about the latest areas of research in sarcoma, explore these related items that take you outside of this guide:

• To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases.

• Listen to a podcast from an ASCO expert discussing highlights in sarcoma research from ASCO Annual Meetings in 2020 and 2021.

• Visit the Cancer.Net Blog to review other research announced at recent scientific meetings and perspectives on a sarcoma diagnosis.

• Visit the website of Conquer Cancer, the ASCO Foundation, to find out how to help support cancer research. Please note that this link takes you to a different ASCO website.

The next section in this guide is Coping with Treatment. It offers some guidance on how to cope with the physical, emotional, social, and financial changes that cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will read about your medical care after cancer treatment is completed and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with sarcoma does not end when active treatment has finished. Your health care team will continue to check that the cancer has not come back, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead.

Cancer rehabilitation may be recommended, and this could mean any of a wide range of services, such as physical therapy, occupational therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible. For patients treated for a sarcoma in an arm or leg, a rehabilitation program after surgery or radiation therapy can help them regain or maintain limb function. Range-of-motion exercises, strengthening exercises, and a program to reduce lymphedema may be recommended. A rehabilitation medicine specialist can help patients receive the most appropriate rehabilitation after treatment. The majority of patients with a sarcoma in an arm or leg can be successfully treated and maintain good limb function. However, when treatment included amputation, services that provide artificial limbs, called prosthetics, and additional mental health support can help manage the adjustment to life following the loss of a limb. Learn more about cancer rehabilitation.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence, which means that the cancer has come back. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will ask specific questions about your health. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the type and stage of cancer first diagnosed and the types of treatment given.

The anticipation before having a follow-up test or waiting for test results may add stress to you or a family member. This is sometimes called “scanxiety.” Learn more about how to cope with this type of stress.

Managing long-term and late side effects

Most people expect to have side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years after treatment has ended. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on your diagnosis, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to discuss any concerns you have about your future physical or emotional health. ASCO offers forms to help keep track of the cancer treatment you received and develop a survivorship care plan when treatment is completed.

This is also a good time to talk with your doctor about who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the care of their primary care doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with them and with all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find some questions to ask your doctor or other members of the health care team, to help you better understand your diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with the health care team is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your care. It may also be helpful to ask a family member or friend to come with you to appointments to help take notes.

Questions to ask after getting a diagnosis

• What type of sarcoma do I have?

• Has this diagnosis been reviewed by a pathologist who is experienced in the diagnosis and classification of sarcoma?

• Can you explain my pathology report (laboratory test results) to me?

• What stage and grade is the sarcoma? What does this mean?

• What does my diagnosis mean?

• Where is the tumor located?

• Should I see a sarcoma specialist?

• Was my case discussed in a tumor board or other multidisciplinary conference? What does this mean?

Questions to ask about choosing a treatment and managing side effects

• What are my treatment options?

• What clinical trials are available for me? Where are they located, and how do I find out more about them?

• What treatment plan do you recommend? Why?

• How much experience do you have treating this specific type of sarcoma?

• What is the goal of each treatment? Is it to eliminate the cancer, help me feel better, or both?

• Can the sarcoma be removed with only surgery?

• What are the possible side effects of this treatment, both in the short term and the long term?

• Who will be part of my health care team, and what does each member do?

• Who will be leading my overall treatment?

• How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

• Could this treatment affect my sex life? If so, how and for how long?

• Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before cancer treatment begins?

• If I’m worried about managing the costs of cancer care, who can help me?

• How can I keep myself as healthy as possible during and after treatment?

• What support services are available to me? To my family?

• If I have questions or problems, who should I call?

Questions to ask about having surgery

• Should I have a biopsy before surgery?

• Does the stage of cancer affect the decision about surgery? How?

• What will happen during this surgery?

• How long will the operation take?

• How long will I be in the hospital?

• Will the surgery be performed as en-bloc resection? What does this mean?

• How much experience does this surgeon have with sarcoma? How many sarcoma operations have they done in the past year?

• What are the possible side effects of my surgery, both in the short term and long term?

• Who should I contact about any side effects I experience? And how soon?

• Do I need chemotherapy and/or radiation therapy before the surgery? After the surgery?

• Can you describe what my recovery from surgery will be like?

• Will I need rehabilitation after the surgery?

• If surgery is on an arm or leg, what will my limb look like after surgery?

Questions to ask about having radiation therapy

• What type of radiation therapy is recommended?

• What is the goal of this treatment?

• How experienced is the radiation oncologist with sarcoma? How many people with sarcoma have they treated in the past year?

• How many treatments will I need, and how long will each one take?

• What are the possible side effects, both in the short term and long term?

• Who should I contact about any side effects I experience? And how soon?

• What can be done to prevent or to treat the side effects?

Questions to ask about having systemic therapy using medication

• What type of treatment is recommended?

• What is the goal of this treatment?

• How experienced is the medical oncologist with sarcoma? How many people with sarcoma have they treated in the past year?

• Are other systemic therapies using medication available?

• Should I consider a clinical trial?

• How many treatments will I need? How long will each take?

• Will I receive this treatment at a hospital or clinic? Or will I take it at home?

• What side effects can I expect during treatment?

• Who should I contact about any side effects I experience? And how soon?

• What are the possible long-term or late effects of having this treatment?

• What can be done to prevent or relieve side effects?

Questions to ask about planning follow-up care

• What is the chance that the cancer will come back? Should I watch for specific signs or symptoms?

• What long-term side effects or late effects are possible based on the cancer treatment I received?

• What follow-up tests will I need, and how often will those tests be needed?

• How do I get a treatment summary and survivorship care plan to keep in my personal records?

• Who will be leading my follow-up care?

• What survivorship support services are available to me? To my family?

The next section in this guide is Additional Resources. It offers more resources on this website that may be helpful to you. Use the menu to choose a different section to read in this guide.