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Sarcomas of Specific Organs - Introduction

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will find some basic information about these diseases and the parts of the body they may affect. This is the first page of Cancer.Net’s Guide to Sarcomas of Specific Organs. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

About sarcoma

Soft-tissue sarcoma (STS) is cancer that develops in the tissues that support and connect the body. A sarcoma can occur in fat tissue, muscles, nerves, tendons, joints, blood vessels, or lymph vessels. A sarcoma begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread.

When a sarcoma is small, it usually does not cause problems and may go unnoticed or appear harmless. Most people with a small STS can be treated successfully. However, if the sarcoma grows, it can interfere with the body's normal activities, and it can spread to other parts of the body. Sarcoma that has spread is more challenging to treat successfully.

Sarcoma can begin in any part of the body. About 60% start in an arm or leg, 30% start in the trunk or abdomen, and 10% start in the head or neck. Sarcoma is uncommon and accounts for about 1% of all cancers.

About sarcomas found in specific organs

Because there are several different types of STS, it is considered a family of related diseases and not a single, specific disease. Sarcomas that are found in a specific organ have different names and are different from other sarcomas. Some of these organ-specific sarcomas are listed below.

Body Part

Specific Sarcoma(s)

Aorta, pulmonary artery, pulmonary vein (large blood vessels coming out of the heart)

Intimal sarcoma

Brain and its coverings, called meninges

Solitary fibrous tumor

Breast

Phyllodes tumor, angiosarcoma

Heart

Angiosarcoma, rhabdomyosarcoma; benign myxomas are more common

Liver

Embryonal sarcoma, epithelioid hemangioendothelioma

Lymph nodes or other lymphatic tissue

Lymphangioleiomyomatosis, perivascular epithelial cell tumor (PEComa), true histiocytic sarcoma, follicular dendritic cell tumor, interdigitating cell tumor

Pleura, the lining around the lungs

Epithelioid hemangioendothelioma, solitary fibrous tumor, synovial sarcoma

Prostate

Rhabdomyosarcoma, prostatic stromal sarcoma

Uterus

Endometrial stromal sarcoma, undifferentiated endometrial sarcoma, adenosarcoma, PEComa

Each of these sarcomas has different characteristics. Some of the sarcomas listed above are relatively slow growing. Others can grow quite quickly. For example, phyllodes tumors can grow quickly but, unlike most common breast cancers, only very rarely spread to lymph nodes.

Some of the rare sarcomas specific to particular organs listed above are discussed in this section. You can learn more about STS in a separate section on Cancer.Net.

Looking for More of an Introduction?

If you would more of an introduction, explore these related items. Please note that these links will take you to another section of Cancer.Net:

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with this disease and general survival rates. You may use the menu to choose a different section to read in this guide.  

Sarcomas of Specific Organs - Statistics

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find information about the number of people who are diagnosed with STS each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors. Use the menu to see other pages.

Overall, sarcoma is uncommon. This year, about 12,750 people (7,240 men and boys and 5,510 women and girls) will be diagnosed with STS in the United States.

An estimated 5,270 people (2,840 men and boys and 2,430 women and girls) are expected to die of the disease this year.

The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The overall 5-year survival rate for people with STS is around 65%. However, rates vary based on the specific type and the stage, or extent, of the sarcoma. The statistics below include different types of STS, but do not include Kaposi sarcoma.

The 5-year survival rate for people with STS that is only in the area it started is around 81%. About 58% of patients are diagnosed at this early stage. If the sarcoma has spread to the nearby lymph nodes or regional tissue, the 5-year survival rate is 58%. If the sarcoma has spread to another area of the body, the 5-year survival rate is 16%. Talk with your doctor about what to expect with your specific diagnosis.

It is important to remember that statistics on the survival rates for STS are an estimate. The estimate comes from annual data based on the number of people with this cancer in the United States. Also, experts measure the survival statistics every 5 years. So the estimate may not show the results of better diagnosis or treatment available for less than 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's (ACS) publication, Cancer Facts and Figures 2017: Special Section – Rare Cancers in Adults, and the ACS website (January 2019).

The next section in this guide is Risk Factors. It explains the factors that may increase the chance of developing STS and what may lower your risk. Use the menu to choose a different section to read in this guide.

 

 

Sarcomas of Specific Organs - Risk Factors

Approved by the Cancer.Net Editorial Board, 08/2017

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

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 a known cause. However, the following factors can raise a person’s risk of developing sarcoma:

  • Previous radiation therapy. People who have received radiation therapy as treatment for a previous cancer have a slightly increased risk of developing a sarcoma later in life. Sometimes it is an angiosarcoma that develops, but osteosarcoma and undifferentiated pleomorphic sarcoma may also be diagnosed.

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

The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems this disease can cause. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. Use the menu to see other pages.

People with sarcoma may experience the following symptoms or signs. Often, the only sign that there is a problem is that body part containing the tumor will get bigger or there will be pain. Sometimes, people with an STS do not have any symptoms. Or, the cause of a symptom may be a different medical condition that is not cancer.

Symptoms of sarcoma may include:

  • A painless mass or growth in the leg, buttock, or sometimes on the face or neck.

  • Pain or soreness in the area of the mass caused by the tumor pushing on nerves or muscles.

  • Limping or other problems with the legs or feet.

  • Stiffness in the area with the tumor or difficulty moving an arm or leg.

For a sarcoma affecting a specific organ, the symptoms are often related to the organ or body part where the sarcoma develops. For example, with cardiac sarcoma, symptoms depend on where the tumor is located. A tumor can be inside the heart’s chambers, in the muscles of the heart, or on the outside of the heart. If the tumor is inside the heart, it can block the flow of blood and cause swelling of the feet, legs, ankles, or abdomen, as well as distention, or stretching, of neck veins. This happens because the blood returning to the heart after traveling through the body cannot easily enter or leave the right atrium, a heart chamber.

Cardiac sarcoma that occurs in the pericardium, which is the membrane outside of the heart, can cause bleeding into and swelling in the pericardial sac. This interferes with the heart’s ability to pump blood. Symptoms may include chest pain, shortness of breath, fatigue, and heart palpitations, which are a forced or irregular heartbeat.

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help figure out the cause of the problem, called a diagnosis.

If sarcoma is diagnosed, relieving symptoms remains an important part of your care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Diagnosis

Approved by the Cancer.Net Editorial Board, 08/2017

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 this happens, it is called metastasis. For example, imaging tests can show if the cancer has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know whether 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.

This list describes options for diagnosing this type of cancer. Not all tests listed below 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 medical condition

  • The results of earlier medical tests

In addition to a physical examination, the following tests may be used to diagnose all types of STS:

  • Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. A pathologist then analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. A biopsy is the most definitive test for diagnosing sarcoma.

  • X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation.

  • Computed tomography (CT or CAT) scan. A CT scan creates a 3-dimensional picture of the inside of the body using x-rays taken from different angles. A computer combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. CT scans can be used to guide a needle biopsy, in which a very thin needle is inserted into the suspicious area and a sample of cells is gathered for examination under a microscope. A CT scan can be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a liquid to swallow.

  • Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. 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 or given as a liquid to swallow.

The following tests may also be used to diagnose a specific type of STS, depending on the parts of the body affected and other factors:

  • Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. 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 gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

  • Blood tests. A complete blood count (CBC) may be done to measure the different types of blood cells.

  • Coronary arteriogram. A coronary arteriogram highlights any abnormalities of the arteries. A dye is injected into an artery and then an x-ray is taken.

  • 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 in the breast. 

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is sarcoma, these results also help the doctor describe it. 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. You may use the menu to choose a different section to read in this guide. 

Sarcomas of Specific Organs - Stages and Grades

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread, as well as the way the sarcoma cells look when viewed under a microscope. This is called the stage and grade. Use the menu to see other pages.

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out a sarcoma's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of cancer.

Sarcomas that begin in organs are not assigned stage groupings. However, they are evaluated using the TNM system described below.

TNM staging system

One tool that doctors use to describe sarcoma is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?

  • Metastasis (M): Has the cancer metastasized to other parts of the body? If so, where and how much?

  • Grade (G): How different are the cancer cells from surrounding healthy cells?

Tumor (T)

Using the TNM system, the "T" plus a number (1 to 4) and letter (a, b, or c) is used to describe the size and location of the tumor. Specific tumor information is listed below.

TX: The primary tumor cannot be evaluated.

T1: The tumor is confined only to the organ where it started.

T2: The tumor has spread into the tissue outside of the organ.

  • T2a: The tumor has grown into the membrane that covers organs, called the serosa, or the lining of the organs in the abdomen, called the visceral peritoneum.

  • T2b: The tumor has grown beyond the serosa.

T3: The tumor has spread to another organ.

T4: The tumor is found in more than 1 place in a particular organ or specific part of the body, called multifocal involvement.

  • T4a: The tumor involves 2 places in a single organ.

  • T4b: The tumor involves 3 to 5 places in a single organ.

  • T4c: The tumor involves more than 5 places in a single organ.

Node (N)

The “N” in the TNM staging system stands for lymph nodes. These tiny, bean-shaped organs help fight infection. Each type of tumor drains into nearby lymph nodes, called regional lymph nodes. Sarcoma rarely spreads to the lymph nodes.

N0 (N plus zero): The cancer has not spread to the regional lymph nodes or it is unknown if lymph nodes are involved.

N1: The cancer has spread to the regional lymph nodes.

Metastasis (M)

The "M" in the TNM system indicates if the cancer has spread to other parts of the body, called distant metastasis.

M0 (M plus zero): The cancer has not metastasized.

M1: There is metastasis to another part or parts of the body.

Grade (G)

As described above, doctors also describe this type of cancer by its grade (G), which describes how much cancer cells look like healthy cells when viewed under a microscope.

The doctor compares the cancerous tissue with healthy tissue. Healthy tissue usually contains many different types of cells grouped together. If the cancer looks similar to healthy tissue and contains different cell groupings, it is called “differentiated” or a “low-grade tumor.” If the cancerous tissue looks very different from healthy tissue, it is called “poorly differentiated” or a “high-grade tumor.” The cancer’s grade may help the doctor predict how quickly the cancer will spread. In general, the lower the tumor’s grade, the better the prognosis.

There are 4 grades for sarcoma: GX (the grade cannot be evaluated), G1, G2, and G3. The grades are determined based on the factors below:

  • Differentiation: How different the cancer cells are from normal tissue cells.

  • Mitotic count: How many tumor cells are dividing.

  • Tumor necrosis: How much of the tumor has cells that are dying.

The lower the combined score for these 3 factors, the lower the grade. A lower grade means that the tumor is less aggressive and that a patient’s prognosis is better. This is the recommended grading system from the French Federation of Cancer Centers Sarcoma Group (French Fédération Nationale des Centres de Lutte Contre le Cancer or FNCLCC), but other systems may also be used. For instance, some doctors grade sarcomas as either “low grade” or “high grade,” particularly if the tumor has been treated before surgery.

Used with permission of the American College of Surgeons, Chicago, Illinois. The original and primary source for this information is the AJCC Cancer Staging Manual, Eighth Edition (2017) published by Springer International Publishing.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Treatment Options

Approved by the Cancer.Net Editorial Board, 08/2017

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

This section tells you the treatments that are the standard of care for this type of sarcoma. “Standard of care” means the best treatments known. When making treatment plan decisions, patients are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Your doctor can help you consider all your treatment options. To learn more about clinical trials, see the About Clinical Trials and Latest Research sections.

Treatment overview

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, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Descriptions of the most common treatment options for sarcomas of specific organs are listed below. Treatment options and recommendations depend on several factors, including the type and stage of the sarcoma, the location of the tumor, possible side effects, and the patient’s preferences and overall health.

Unfortunately, there are so many different types of sarcoma that it is not possible to describe the best treatments for each of the rare sarcomas in this section. It is strongly recommended that people who are diagnosed with a rare type of sarcoma talk with a doctor at sarcoma expert centers. There may be new drugs in clinical trials that may be the best option for treatment. Talk with your doctor about finding a specialist center.

Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue, called the margin, during an operation. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

Surgery is the most common treatment for most sarcomas that develop in a specific organ. If the tumor is in an arm or leg, surgical techniques can often be used to avoid removing the limb. However, because the surgeon may need to take a wide margin of tissue to make sure no cancer remains, occasionally there may be a need to remove part or all of a limb, called amputation. If amputation is needed, rehabilitation that includes physical therapy can help a patient have the most physical function possible. Rehabilitation can also help a person cope with the social and emotional effects of losing a limb.

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

Certain types of sarcoma cannot be removed using surgery. For example, epithelioid hemangioendothelioma of the liver usually affects many parts of the liver at once and other parts of the body. As a result, surgery, even liver transplantation, cannot completely eliminate the cancer. This is similar to the situation for 80% of people with cardiac sarcoma. By the time the cardiac tumor causes symptoms, it has already spread and cannot be completely removed with surgery. In these situations, radiation therapy or chemotherapy will typically be recommended instead (see below).

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

Radiation therapy may be used before surgery to shrink the size of the tumor or after surgery to destroy any remaining cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.

Learn more about the basics of radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by ending the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication.

Systemic chemotherapy gets into the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

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 combinations of different drugs given at the same time.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.

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

Kinase inhibitors (updated 01/2019)

Kinase inhibitors are a type of targeted therapy that blocks the function of a protein found in sarcoma cells. This type of drug blocks the growth and spread of cancer cells while limiting damage to healthy cells.

Pazopanib (Votrient) is an approved targeted therapy for sarcomas that are not stopped by other treatment options. Pazopanib is not used for liposarcoma or GIST. The drug starves sarcoma cells of a blood supply, which helps slow tumor growth and sometimes shrinks sarcomas. The most common side effects of pazopanib are diarrhea, fatigue, loss of coloration in the hair and skin, and high blood pressure. Talk with your doctor about possible side effects for a specific medication and how they can be managed. Learn more about the basics of targeted treatments.

A small percentage of sarcomas, less than 1%, have a mutation in the neurotrophic receptor tyrosine kinase (NTRK) gene. Larotrectinib (Vitrakvi) is an NTRK inhibitor that is now approved for any cancer that has a specific mutation in the NTRK gene. The most common side effects include fatigue, nausea, dizziness, vomiting, increased liver enzymes, cough, constipation, and diarrhea.

Clinical trials are taking place to find out more about new treatments for rare sarcomas unique to specific body parts. See the Latest Research section for more information.

Organ transplantation

Organ transplantation involves replacing an organ affected by sarcoma with a healthy organ from a donor. For example, heart transplantation may be used as a treatment for cardiac sarcoma, and liver transplantation may be used to treat a sarcoma that is only growing in the liver.

For a transplant to be successful, the patient will have to take immunosuppressive medication to help the patient’s body accept the new organ. As a result of taking this medication, the patient could develop a new cancer or the sarcoma might come back. In addition, people may have to wait a long time for a donor organ to become available. Therefore, patients and their doctors should carefully consider and talk about this treatment option.

Immunotherapy (updated 01/2019)

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Most immunotherapy treatments involve “immune checkpoint inhibitors.” These drugs are given to take the brakes off the body’s natural immune response against the cancer in the body. The current methods of immunotherapy do have problems because these drugs also activate immune responses against normal body parts, a process called autoimmunity. Some of these drugs are approved to treat other cancers. They are not approved in sarcomas because they have not been tested well enough yet.

However, if testing on your tumor shows that it has specific problems with repairing DNA damage, called microsatellite instability-high (MSI-H) or mismatch repair deficiency (dMMR; this occurs in less than 1% of sarcomas), then a checkpoint inhibitor called pembrolizumab (Keytruda) may be used.

In uncommon situations, white blood cells can be trained to fight cancer. If a specific target can be identified in your cancer, specially engineered T-cells from your own body are used to attack the cancer cells. This kind of immunotherapy is called chimeric antigen receptor (CAR) T-cell therapy. At present, these cellular treatments are given only in clinical trials for people with specific types of sarcoma.

Learn more about the basics of immunotherapy.

Getting care for symptoms and side effects

Sarcoma and its treatment often cause side effects. In addition to treatments intended to slow, stop, or eliminate the tumor, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process. People often receive treatment for the tumor at the same time that they receive treatment to ease side effects. In fact, patients who receive both at the same time often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the sarcoma, such as chemotherapy, surgery, or radiation therapy. Talk with your doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and palliative care options. During and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.

Metastatic sarcoma

If sarcoma spreads to another part in the body from where it started, doctors call it metastatic sarcoma. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Also, clinical trials might be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.

Your treatment plan may include a combination of chemotherapy, radiation therapy, and possibly surgery. Palliative care will also be important to help relieve symptoms and side effects.

For most patients, a diagnosis of metastatic sarcoma is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission can be temporary or permanent. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the sarcoma returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the sarcoma does return. Learn more about coping with the fear of recurrence.

If the sarcoma does return after the original treatment, it is called recurrent sarcoma. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the treatments described above, such as surgery, chemotherapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat this type of recurrent sarcoma. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent sarcoma often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

If treatment doesn’t work

Recovery from sarcoma is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and for many people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced cancer and who are expected to live less than 6 months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

The next section in this guide is About Clinical Trials and it offers more information about research studies that are focused on finding better ways to care for people with cancer. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. Use the menu to see other pages.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for patients with sarcomas that are specific to particular organs. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials can be some of the first to get a treatment before it is available to the public. However, there are some risks with a clinical trial, including possible side effects and that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects. There are also clinical trials studying ways to prevent cancer.

Deciding to join a clinical trial

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are a way to contribute to the progress in rare cancers such as sarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with sarcoma.

Insurance coverage of clinical trials costs differs by location and by study. In some programs, some of the patient’s expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how your treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” Placebos are usually combined with standard treatment in most cancer clinical trials. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should:

  • Describe all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different from the risks of standard treatment.

  • Explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep patients safe. You and the research team will carefully review these criteria together.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for sarcoma, learn more in the Latest Research section.

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of tumor.

PRE-ACT, Preparatory Education About Clinical Trials

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

The next section in this guide is Latest Research. It explains areas of scientific research currently going on for this type of cancer. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Latest Research

Approved by the Cancer.Net Editorial Board, 08/2017

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

Doctors are working to learn more about sarcoma, ways to prevent them, how to best treat these diseases, and how to provide the best care to people diagnosed with either 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.

  • Targeted therapy. As described in Treatment Options, 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 new targeted drug, pazopanib. Clinical trials of other drugs that work in different ways to starve tumors of their blood supply are also underway.

  • Immunotherapy. As noted in Treatment Options, immunotherapy is being researched as a treatment option for many types of sarcoma. Learn more about immunotherapy.

  • Chemotherapy. New types of chemotherapy are being researched for the treatment of sarcoma. Many of these drugs focus on ways to treat sarcoma that have never been tested before.

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

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding sarcomas of specific organs, explore these related items that take you outside of this guide:

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

Sarcomas of Specific Organs - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of cancer and its treatment. This page includes several links outside of this guide to other sections of this website. Use the menu to see other pages.

Every cancer treatment can cause side effects or changes to your body and how you feel. For many reasons, people don’t experience the same side effects even when they are given the same treatment for the same type of cancer. This can make it hard to predict how you will feel during treatment.

As you prepare to start cancer treatment, it is normal to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. Doctors call this part of cancer treatment “palliative care.” It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for sarcoma are described in the Treatment Options section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the cancer’s stage, the length and dose of treatment, and your general health.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

Coping with emotional and social effects

You can have emotional and social effects as well as physical effects after a cancer diagnosis. This may include dealing with difficult emotions, such as sadness, anxiety, or anger, or managing your stress level. Sometimes, patients have problems expressing how they feel to their loved ones, or people don’t know what to say in response.

Patients and their families are encouraged to share their feelings with a member of their health care team. You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

Coping with financial effects

Cancer treatment can be expensive. It is often a big source of stress and anxiety for people with cancer and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost stops them from following or completing their cancer treatment plan. This can put their health at risk and may lead to higher costs in the future. Patients and their families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations in a separate part of this website.

Caring for a loved one with cancer

Family members and friends often play an important role in taking care of a person with sarcoma. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away.

Caregivers may have a range of responsibilities on a daily or as-needed basis. Below are some of the responsibilities caregivers take care of:

  • Providing support and encouragement

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

Learn more about caregiving.

Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they are likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you don’t think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care you may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan.

The next section in this guide is Follow-up Care. It explains the importance of checkups after you finish cancer treatment. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Follow-Up Care

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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 cancer doesn’t end when active treatment has finished. Your health care team will continue to check to make sure the cancer has not returned, 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. Typical follow-up care for a person treated for sarcoma can include a yearly chest x-ray, CT scan, and blood tests. Patients who undergo surgery for sarcoma located in an arm or leg often need physical therapy and other types of rehabilitation. Follow-up care should also address the patient’s quality of life, including social and emotional concerns. This is especially true if a patient’s treatment required an amputation. 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. 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 originally diagnosed and the types of treatment given.

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

Managing long-term and late side effects

Most people expect to experience 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 afterwards. 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 the type of cancer, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may 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 create a treatment summary to 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 decide who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the care of their family 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 him or her 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. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Survivorship

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to see other pages.

What is survivorship?

The word “survivorship” means different things to different people. Common definitions include:

  • Having no signs of cancer after finishing treatment.

  • Living with, through, and beyond cancer. According to this definition, cancer survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

Survivorship is one of the most complicated parts of having cancer. This is because it is different for everyone.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain about coping with everyday life.

Survivors may feel some stress when their frequent visits to the health care team end after completing treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and people miss this source of support. This may be especially true when new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexuality and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing

  • Thinking through solutions

  • Asking for and allowing the support of others

  • Feeling comfortable with the course of action you choose

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the place where you received treatment.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the cancer diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving in this article.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make positive lifestyle changes.

People recovering from sarcoma are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

It is important to have recommended medical checkups and tests (see Follow-up Care) to take care of your health. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical 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.

Talk with your doctor to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note that these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: Get this 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The booklet is available as a PDF, so it is easy to print out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes next after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including those in different age groups.

The next section offers Questions to Ask the Health Care Team to help start conversations with your cancer care team. You may use the menu to choose a different section to read in this guide.  

Sarcomas of Specific Organs - Questions to Ask the Doctor

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will find some questions to ask your doctor or other members of your 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 your 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.

Questions to ask after getting a diagnosis

  • What type of sarcoma do I have?

  • Where exactly is the tumor located?

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

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

Questions to ask about choosing a treatment and managing side effects

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

  • 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?

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

  • Is surgery an option to remove the sarcoma?

  • Do you recommend any additional treatment?

  • 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?

  • Whom should I call with questions or problems?

  • Is there anything else I should be asking?

Questions to ask about having surgery

  • How long will the operation take?

  • How long will I be in the hospital?

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

  • What are the possible long-term effects of having this surgery?

  • Will you recommend specific cancer rehabilitation services for me after treatment?

Questions to ask about having radiation therapy, chemotherapy, or targeted therapy

  • What type of treatment is recommended?

  • What is the goal of this treatment?

  • How long will it take to give this treatment?

  • What side effects can I expect during treatment?

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

  • What can be done to relieve the 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 I need them?

  • 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 some more resources on this website beyond this guide that may be helpful to you. You may use the menu to choose a different section to read in this guide.

Sarcomas of Specific Organs - Additional Resources

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Sarcomas of Specific Organs. Use the menu to go back and see other pages.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Beyond this guide, here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Sarcomas of Specific Organs. You may use the menu to choose a different section to read in this guide.