Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium.

The endometrium is the innermost lining of the uterus. The uterus is a hollow, muscular organ in a woman’s pelvis. The uterus is where a fetus grows. In most nonpregnant women, the uterus is about 3 inches long.

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Cancer of the endometrium is different from cancer of the muscle of the uterus, which is called uterine sarcoma. For more information, visit Uterine Sarcoma Treatment.

Other PDQ summaries containing information related to endometrial cancer include:

Endometrial cancer is most common in postmenopausal women.

Endometrial cancer occurs most often in postmenopausal women, with 60 being the average age at diagnosis.

From 2012 to 2021, the number of new cases of endometrial cancer increased slightly in White women and by 2% to 3% each year in women of all other racial and ethnic groups. From 2013 to 2022, the number of deaths from endometrial cancer increased by just under 2% each year.

Avoiding risk factors and increasing protective factors may help prevent cancer.

Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, having overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer.

The following risk factors increase the risk of endometrial cancer:

The following protective factors decrease the risk of endometrial cancer:

It is not known if the following factors affect the risk of endometrial cancer:

Cancer prevention clinical trials are used to study ways to prevent cancer.

Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials include healthy people who may or may not have an increased risk of cancer. Other prevention trials include people who have had cancer and are trying to prevent recurrence or a second cancer.

The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements.

New ways to prevent endometrial cancer are being studied in clinical trials.

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.

About PDQ

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.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about endometrial cancer prevention. 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.

Reviewers and Updates

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 Screening and Prevention 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Screening and Prevention Editorial Board. PDQ Endometrial Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/uterine/patient/endometrial-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389201]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium.

The endometrium is the lining of the uterus, a hollow, muscular organ in a woman’s pelvis. The uterus is where a fetus grows. In most nonpregnant women, the uterus is about 3 inches long. The lower, narrow end of the uterus is the cervix, which leads to the vagina.

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Cancer of the endometrium is different from cancer of the muscle of the uterus, which is called sarcoma of the uterus. See the PDQ summary on Uterine Sarcoma Treatment for more information about uterine sarcoma.

Obesity and having metabolic syndrome may increase the risk of endometrial cancer.

Taking tamoxifen for breast cancer or taking estrogen alone (without progesterone) can increase the risk of endometrial cancer.

Endometrial cancer may develop in breast cancer patients who have been treated with tamoxifen. A patient who takes this drug and has abnormal vaginal bleeding should have a follow-up exam and a biopsy of the endometrial lining if needed. Women taking estrogen (a hormone that can affect the growth of some cancers) alone also have an increased risk of endometrial cancer. Taking estrogen combined with progesterone (another hormone) does not increase a woman’s risk of endometrial cancer.

Signs and symptoms of endometrial cancer include unusual vaginal bleeding or pain in the pelvis.

These and other signs and symptoms may be caused by endometrial cancer or by other conditions. Check with your doctor if you have any of the following:

Tests that examine the endometrium are used to diagnose endometrial cancer.

Because endometrial cancer begins inside the uterus, it does not usually show up in the results of a Pap test. For this reason, a sample of endometrial tissue must be removed and checked under a microscope to look for cancer cells. One of the following procedures may be used:

Other tests and procedures used to diagnose endometrial cancer include the following:

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

Endometrial cancer can usually be cured because it is usually diagnosed early.

After endometrial cancer has been diagnosed, tests are done to find out if cancer cells have spread within the uterus or to other parts of the body.

The process used to find out whether the cancer has spread within the uterus or to other parts of the body is called staging. 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. Certain tests and procedures are used in the staging process. A hysterectomy (an operation in which the uterus is removed) will usually be done to treat endometrial cancer. Tissue samples are taken from the area around the uterus and checked under a microscope for signs of cancer to help find out whether the cancer has spread.

The following procedures may be used in the staging process:

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 endometrial cancer spreads to the lung, the cancer cells in the lung are actually endometrial cancer cells. The disease is metastatic endometrial cancer, not lung cancer.

The following stages are used for endometrial cancer:

Stage I

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In stage I, cancer is found in the uterus only. Stage I is divided into stages IA and IB, based on how far the cancer has spread.

• Stage IA: Cancer is in the endometrium only or less than halfway through the myometrium (muscle layer of the uterus).
• Stage IB: Cancer has spread halfway or more into the myometrium.

Stage II

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In stage II, cancer has spread into connective tissue of the cervix, but has not spread outside the uterus.

Endometrial cancer may be grouped for treatment as follows:

Endometrial cancer can recur (come back) after it has been treated.

The cancer may come back in the uterus, the pelvis, in lymph nodes in the abdomen, or in other parts of the body.

There are different types of treatment for patients with endometrial cancer.

Different types of treatment are available for patients with endometrial cancer. 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:

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for endometrial cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit 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.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

About PDQ

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.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of endometrial cancer. 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.

Reviewers and Updates

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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Endometrial Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/uterine/patient/endometrial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389334]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Ewing sarcoma is a type of cancer that forms from a certain kind of cell in bone or soft tissue.

Ewing sarcoma may form in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull. It may also form in the body’s soft tissues, which connect, support, and surround other body parts and organs.

Ewing sarcoma is most common in adolescents and young adults (teens through mid-20s).

Ewing sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of the chest wall), extraosseous Ewing sarcoma (Ewing sarcoma in tissue other than bone), and Ewing sarcoma family of tumors.

Undifferentiated small round cell sarcoma may also form in the bone or soft tissue.

Undifferentiated small round cell sarcoma usually forms in the bones or the muscles that are attached to bones and that help the body move. Undifferentiated small round cell sarcoma is usually treated the same way as Ewing sarcoma. Types of undifferentiated small round cell sarcomas include:

A genetic condition may increase the risk of Ewing sarcoma and other sarcomas.

Ewing sarcoma is caused by certain changes to the way bone cells function, especially how they grow and divide into new cells. Often, the exact cause of these changes is unknown. Learn more about how cancer develops at What Is Cancer?

A risk factor is anything that increases the chance of getting a disease. Not every child with a risk factor will develop Ewing sarcoma or other sarcomas. And they can develop in some children who don’t have a known risk factor. Children with Fanconi anemia may be at increased risk of Ewing sarcoma. Talk with your child’s doctor if you think your child may be at risk.

Symptoms of Ewing sarcoma include swelling and pain near the tumor.

It’s important to check with your child’s doctor if your child has:

These symptoms may be caused by problems other than Ewing sarcoma. The only way to know is for your child to see a doctor.

Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma.

If your child has symptoms that suggest Ewing sarcoma, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They also will ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with Ewing sarcoma, the results of these tests will help you and your child’s doctor plan treatment.

Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. To plan treatment, it is important to know whether the cancer has spread to other parts of the body. Tests and procedures to detect, diagnose, and stage Ewing sarcoma are usually done at the same time.

The tests used to diagnose and stage Ewing sarcoma may include:

Certain factors affect prognosis (chance of recovery).

If your child has been diagnosed with Ewing sarcoma, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis. The factors that affect prognosis are different before and after treatment.

Before any treatment is given, prognosis depends on:

After treatment is given, prognosis is affected by:

If the cancer recurs after initial treatment, prognosis depends on:

Ewing sarcoma is described as localized, metastatic, or recurrent.

Children with Ewing sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of Ewing sarcoma. The pediatric oncologist works with other health care providers who are experts in treating children with Ewing sarcoma and who specialize in certain areas of medicine. Other specialists may include:

There are different types of treatment for children with Ewing sarcoma.

You and your child’s care team will work together to decide treatment. Many factors will be considered, such as where the cancer is located, your child’s age and overall health, and whether the cancer is newly diagnosed or has come back.

Your child’s treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s care team before treatment begins about what to expect. For help every step of the way, visit our booklet, Children with Cancer: A Guide for Parents.

The following types of treatment are used:

You may want to think about having your child take part in a clinical trial.

For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.

You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Treatment for Ewing sarcoma may cause side effects.

Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the effects cancer treatment can have on your child. Learn more about Late Effects of Treatment for Childhood Cancer.

Follow-up care may be needed.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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 child’s condition has changed or if the cancer has recurred (come back).

To learn more about these follow-up tests, visit Tests to diagnose Ewing sarcoma.

About PDQ

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.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood Ewing 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.

Reviewers and Updates

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 Pediatric 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Ewing Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/bone/patient/ewing-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389350]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Kaposi sarcoma is a disease in which malignant lesions (cancer) can form in the skin, mucous membranes, lymph nodes, and other organs.

Kaposi sarcoma is a cancer that causes lesions (abnormal tissue) to grow in the skin; the mucous membranes lining the mouth, nose, and throat; lymph nodes; or other organs. The lesions are usually purple and are made of cancer cells, new blood vessels, red blood cells, and white blood cells. Kaposi sarcoma is different from other cancers in that lesions may begin in more than one place in the body at the same time.

Human herpesvirus-8 (HHV-8) is found in the lesions of all patients with Kaposi sarcoma. This virus is also called Kaposi sarcoma herpesvirus (KSHV). Most people with HHV-8 do not get Kaposi sarcoma. People with HHV-8 are more likely to develop Kaposi sarcoma if their immune system is weakened by disease, such as human immunodeficiency virus (HIV), or by drugs given after an organ transplant.

There are several types of Kaposi sarcoma. The two types discussed in this summary include:

Tests that examine the skin, lungs, and gastrointestinal tract are used to diagnose Kaposi sarcoma.

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

After Kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The following tests and procedures may be used to find out if cancer has spread to other parts of the body:

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

Classic Kaposi sarcoma is found most often in older men of Italian or Eastern European Jewish origin.

Classic Kaposi sarcoma is a rare disease that gets worse slowly over many years.

Signs of classic Kaposi sarcoma may include slow-growing lesions on the legs and feet.

Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions may form in other parts of the body, such as the stomach, intestines, or lymph nodes. The lesions usually don’t cause any symptoms but may grow in size and number over a period of 10 years or more. Pressure from the lesions may block the flow of lymph and blood in the legs and cause painful swelling. Lesions in the digestive tract may cause gastrointestinal bleeding.

Another cancer may develop.

Some patients with classic Kaposi sarcoma may develop another type of cancer before the Kaposi sarcoma lesions appear or later in life. Most often, this second cancer is non-Hodgkin lymphoma. Frequent follow-up is needed to watch for these second cancers.

Patients with HIV are at risk of developing epidemic Kaposi sarcoma (HIV-associated Kaposi sarcoma).

AIDS is caused by HIV, which attacks and weakens the body’s immune system. A weakened immune system is unable to fight infection and disease. People with HIV have an increased risk of infection and cancer.

A person with HIV and certain types of infection or cancer, such as Kaposi sarcoma, is diagnosed as having AIDS. Sometimes, a person is diagnosed with AIDS and epidemic Kaposi sarcoma at the same time.

The use of drug therapy called highly active antiretroviral therapy (HAART) reduces the risk of epidemic Kaposi sarcoma in patients with HIV.

HAART is a combination of several drugs used to lessen the damage to the immune system caused by HIV infection. Treatment with HAART reduces the risk of epidemic Kaposi sarcoma, although it is possible for a person to develop epidemic Kaposi sarcoma while taking HAART.

For information about AIDS and its treatment, see the HIVinfo website.

Signs of epidemic Kaposi sarcoma can include lesions that form in many parts of the body.

The signs of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following:

Kaposi sarcoma is sometimes found in the lining of the mouth during a regular dental check-up.

In most patients with epidemic Kaposi sarcoma, the disease will spread to other parts of the body over time.

There are different types of treatment for patients with Kaposi sarcoma.

Different types of treatments are available for patients with Kaposi 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.

The following types of treatment are used to treat Kaposi sarcoma:

Treatment of epidemic Kaposi sarcoma combines treatment for Kaposi sarcoma with treatment for acquired immunodeficiency syndrome (AIDS). The types of treatment used to treat Kaposi sarcoma include:

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Treatment for Kaposi sarcoma may cause side effects.

For information about side effects caused by treatment for cancer, visit 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.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

About PDQ

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.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of Kaposi 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.

Reviewers and Updates

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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Kaposi Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389178]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body.

Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following:

Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.

Enlarge

Soft tissue sarcoma occurs in children and adults.

Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.)

Having certain diseases and inherited disorders can increase the risk of childhood 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 child’s doctor if you think your child may be at risk.

Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:

Other risk factors include the following:

The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body.

A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness.

Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems.

Diagnostic tests are used to diagnose childhood soft tissue sarcoma.

The following tests and procedures may be used:

If tests show there may be a soft tissue sarcoma, a biopsy is done.

The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used:

If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery.

To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and 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 and mid-grade tumors usually grow and spread more quickly than low-grade tumors.

Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma.

One or more of the following laboratory tests may be done to study the tissue samples:

There are many different types of soft tissue sarcomas.

The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed.

This summary is about the following types of soft tissue sarcoma:

See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary:

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread 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. There is no standard staging system for childhood soft tissue sarcoma.

To plan treatment, it is important to know the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body.

The following procedures may be used to find out if cancer has spread:

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 soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer.

Sometimes childhood soft tissue sarcoma continues to grow or comes back after treatment.

Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.

Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body.

There are different types of treatment for patients with childhood soft tissue sarcoma.

Different types of treatments are available for patients with childhood 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.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included:

Seven types of standard treatment are used:

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Treatment for childhood soft tissue sarcoma may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

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.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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 child’s condition has changed or if the cancer has recurred (come back).

Fat Tissue Tumors

Bone and Cartilage Tumors

Fibrous (Connective) Tissue Tumors

Skeletal Muscle Tumors

Smooth Muscle Tumors

So-called Fibrohistiocytic Tumors

Nerve Sheath Tumors

Pericytic (Perivascular) Tumors

Tumors of Unknown Cell Origin (the place where the tumor first formed is not known)

Undifferentiated/Unclassified Sarcoma

Blood Vessel Tumors

Metastatic Childhood Soft Tissue Sarcoma

Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following:

For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section.

About PDQ

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.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood 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.

Reviewers and Updates

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 Pediatric 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Incidence and Mortality

Estimated new cases and deaths from soft tissue sarcoma in the United States in 2025:[1]

Risk Factors

Most soft tissue sarcomas are sporadic. Risk factors include:[4–6]

Soft tissue sarcomas occur more frequently in patients with the following inherited syndromes:[4–6]

Clinical Features

Soft tissue sarcomas are a heterogenous family of malignant tumors that may arise in nearly any organ system. The anatomical distribution in adults is as follows:[5]

Diagnostic Evaluation

Adequate tissue should be obtained via either image-guided core-needle biopsy or planned incisional biopsy (for select cases). The samples should be reviewed by a pathologist who is experienced in diagnosing sarcomas. Careful planning of the initial biopsy, with consultation among the surgeon, radiation oncologist, and interventional radiologist, is important to avoid compromising subsequent curative resection. In general, incisional biopsies are reserved for patients whose prior core-needle biopsies were nondiagnostic or when a core-needle biopsy cannot be safely performed because of anatomical constraints.

Before any intervention is initiated, imaging is performed to evaluate the sarcoma and determine if there are metastases. The following modalities may be used as clinically indicated:

Prognostic Factors

Poor prognostic factors in adults with soft tissue sarcomas include:[4–15]

Follow-Up Testing

PET and CT imaging may have higher sensitivity than contrast-enhanced CT imaging when recurrent sarcoma is suspected. Late recurrences (more than 5 years from initial diagnosis) are seen with some histologies, such as synovial sarcoma or alveolar soft-part sarcoma.[17,18]

Evidence (posttreatment imaging surveillance):

This study supports imaging surveillance for detection of lung metastases. Local recurrences at the primary site were usually detected by clinical examination. The impact of metastases detection on overall survival or quality of life is unknown.[19]

Staging Evaluation

In addition to histology, identifying the location and extent of disease (e.g., localized, locally advanced, metastatic) is important in determining the most effective initial treatment for patients with soft tissue sarcoma.[1]

Imaging tests used in the staging evaluation may include:[1–5]

Some staging evaluation procedures depend on the tumor histology and site, including:

Knowledge of intracompartmental or extracompartmental extension of extremity sarcomas is important for surgical decision making. For complete staging, a thorough review of all biopsy specimens, including those from the primary tumor, lymph nodes, or other suspicious lesions, is essential. Nodal involvement is rare, occurring in less than 3% of patients with sarcoma, but it occurs more often in certain subtypes, such as rhabdomyosarcoma, angiosarcoma, synovial sarcoma, clear cell sarcoma, and epithelioid sarcoma.[7,8]

American Joint Committee on Cancer (AJCC) Staging System

The 2017 AJCC/Union for International Cancer Control (UICC) cancer staging classification system recommends the use of the three-tiered French Federation of Comprehensive Cancer Centers (Fédération Nationale des Centres de Lutte Contre Le Cancer [FNCLCC]) Sarcoma Group grading schema. Prognostic factors required for stage grouping are from FNCLCC. The definitions of grade are provided in Table 6. Of note, staging is primarily used as a research tool and does not routinely impact decision making outside of the factors listed above (sarcoma subtype and grade, primary location, extent of disease [localized, locally advanced, distant metastases]).

The AJCC staging system has designated stage by the following criteria:[1–5]

For information on unusual histologies and sites, see the eighth edition of the AJCC Cancer Staging Manual,[1] which indicates that the TNMG staging classification has different T staging criteria and prognostic groups based on the primary tumor site (see Table 1). The characteristic molecular markers of some sarcomas are not formally incorporated in the staging system pending further evaluation of their impact on prognosis.[2–5]

Recurrent sarcomas are restaged using the same system that is used for primary tumors, with the notation that the tumor is recurrent.

Planning Therapy

Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with soft tissue sarcoma. In most cases, a combined modality approach of preoperative radiation therapy (preRT) or postoperative radiation therapy (PORT) is used for treatment, rather than a radical surgical procedure such as amputation. Surgery without PORT may be possible in selected cases. The role of chemotherapy is not well defined.

Treatment Options for Soft Tissue Sarcoma

Surgery

Surgical resection is the mainstay of therapy for soft tissue sarcomas.

In some small low-grade tumors of the extremities or trunk, surgery alone can be performed without the use of radiation. Evidence for this approach is limited to single-institution, relatively small case series [3–5] or analysis of outcomes in the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program tumor registry.[6] These comparisons suffer from low statistical power and differential evaluability rates that could have introduced bias.[3]

Patient selection factors may vary among surgeons. In general, surgery alone is considered in patients with low-grade tumors of the extremity or superficial trunk that are 5 cm or smaller in diameter (T1) and have microscopically negative surgical margins. In these patients, the rate of long-term local tumor control is about 90%.[7]

Radiation Therapy

A patterns-of-care study using SEER data was queried to identify patients undergoing surgery for trunk and extremity soft tissue sarcomas from 2004 to 2009.[27] Of 5,075 patients, 50% received radiation therapy. Radiation therapy was not given as recommended in a significant portion of patients undergoing treatment for soft tissue sarcoma in the United States. Although routine radiation therapy is not recommended for patients with stage I disease, 25% of them still underwent radiation. Even though routine radiation therapy is recommended for patients with stage II and III tumors, only 60% of them underwent radiation. The multivariate analysis identified predictors of radiation therapy efficacy:[27][Level of evidence C2]

Patients with stage III soft tissue sarcoma who received radiation therapy showed improved disease-specific survival at 5 years, compared with those who did not receive radiation therapy (68% vs. 46%, P < .001).

Sometimes the initial management of soft tissue sarcoma cannot include surgical excision because the morbidity would be unacceptable, or nearby critical organs make complete resection impossible. In such circumstances, radiation has been used as the primary therapy;[28] however, this must be considered a treatment of last resort. Experience of radiation as the primary therapy is limited to retrospective case series from single centers.[28][Level of evidence C3]

Chemotherapy

Treatment Options for Stage I Soft Tissue Sarcoma

Treatment options for stage I soft tissue sarcoma include:

For more information on surgery and radiation therapy, see the Treatment Option Overview for Soft Tissue Sarcoma section.

Because of the low metastatic potential of these tumors, chemotherapy is usually not given to patients with stage I soft tissue sarcoma.[1,2]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Stage II and Node-Negative Stage III Soft Tissue Sarcoma

Treatment options for stage II and node-negative stage III soft tissue sarcoma include:

For more information on surgery, radiation therapy, and chemotherapy, see the Treatment Option Overview for Soft Tissue Sarcoma section.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Advanced Stage III (N1) Soft Tissue Sarcoma

Treatment options for advanced stage III (N1) soft tissue sarcoma include:

For more information on surgery, radiation therapy, and chemotherapy, see the Treatment Option Overview for Soft Tissue Sarcoma section.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Stage IV Soft Tissue Sarcoma

Treatment options for stage IV soft tissue sarcoma include:

For more information on surgery and chemotherapy, see the Treatment Option Overview for Soft Tissue Sarcoma section.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Recurrent Soft Tissue Sarcoma

Treatment options for recurrent soft tissue sarcoma include:

For more information on surgery, radiation therapy, and chemotherapy, see the Treatment Option Overview for Soft Tissue Sarcoma section.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of soft tissue sarcoma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Soft Tissue Sarcoma Treatment are:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for 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: /types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389481]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.