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Sarcomas of Specific Organs - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

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. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About sarcoma

Soft tissue sarcoma 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 normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor 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 soft tissue sarcoma 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. This makes it more challenging to treat successfully.

Sarcoma can begin in any part of the body. About 50% start in an arm or leg, 40% start in the trunk or abdomen, and 10% start in the head or neck. Sarcoma is uncommon, accounting for about 1% of all cancers.

About sarcomas unique to particular organs

Because there are several different types of soft tissue sarcoma, it is considered a family of related diseases, rather than a single, specific disease. Some of the sarcomas unique to particular organs are listed below.

Body Part

Unique 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, while others can grow quite quickly. For example, phyllodes tumors can grow quickly but do not travel to lymph nodes like most common breast cancers.

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

Looking for More of an Overview?

If you would like additional introductory information, view a short video led by an ASCO expert in sarcoma that provides basic information and areas of research. Please note this link will take you to another section of Cancer.Net.

To continue reading this guide, use the menu on the side of your screen to select another section.

Sarcomas of Specific Organs - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will find information about how many people are diagnosed with a soft tissue sarcoma each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

This year, approximately 11,930 people (6,610 males and 5,320 females) will be diagnosed with soft-tissue sarcoma in the United States. An estimated 4,870 adults and children (2,600 males and 2,270 females) are expected to die of the disease this year. ASPS and cardiac sarcoma each make up less than 1% of sarcoma cases.

Cancer statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer, so the actual risk for a particular individual may be different. It is not possible to tell a person how long he or she will live with ASPS or cardiac sarcoma. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's publication, Cancer Facts and Figures 2015.

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Sarcomas of Specific Organs - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a soft tissue sarcoma. To see other pages, use the menu on the side of your screen.

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. However, 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.

Genetics. People with certain inherited diseases, such as neurofibromatosis type I, Gardner syndrome, Werner syndrome, tuberous sclerosis syndrome, nevoid basal cell carcinoma syndrome, Li-Fraumeni syndrome, and a specific form of retinoblastoma, have a higher risk of sarcoma.

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, most sarcoma is not known to be associated with specific environmental hazards.

To continue reading this guide, use the menu on the side of your screen to select another section.  

Sarcomas of Specific Organs - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

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 these types of tumors do not have any symptoms. Or, these symptoms may be caused by a medical condition that is not cancer.

Symptoms of soft tissue 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 they develop. 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 (stretching) of neck veins. This happens because the blood returning to the heart after traveling through the body is not able to easily enter or be pumped out of the right atrium, one of the chambers of the heart.

Cardiac sarcoma that occurs in the pericardium, which is the membrane outside of the heart, can cause 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 one or more of the symptoms or signs on this list or other unusual problems with your health, 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 find 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 symptoms you experience, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose sarcoma and find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether a cancerous tumor has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

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

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. The sample removed during the biopsy is analyzed by a pathologist. 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 three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. CT scans can also be used to guide a needle biopsy, in which a fine needle is inserted into the suspicious area and a sample of cells is gathered for microscopic examination. A CT scan can also 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 also 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 soft tissue sarcoma, 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.

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

Coronary arteriogram. During a coronary arteriogram, a dye is injected into an artery and then an x-ray is taken. This test highlights any abnormalities of the arteries.

Mammography. This 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 helps explain the different stages for soft tissue sarcoma. Use the menu on the side of your screen to select Stages and Grades, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - Stages and Grades

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

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. To see other pages, use the menu on the side of your screen.

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.

One tool that doctors use to describe the stage is the TNM system. TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of a tumor:

  • How large is the primary tumor and where is it located? (Tumor, T)
  • Has the tumor spread to the lymph nodes? (Node, N)
  • Has the tumor metastasized to other parts of the body? (Metastasis, M)

When staging sarcoma, another factor, grade (G), is added to the TNM system. This is different from staging for many other types of cancer.

The results are combined to determine the stage for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the tumor, so doctors can work together to plan the best treatments.

Here are more details on each part of the TNM system for soft tissue sarcoma. This staging system applies to all types of soft tissue sarcoma except Kaposi sarcoma, gastrointestinal stromal tumor (GIST), and infantile fibrosarcoma. Also excluded from this staging system are sarcomas that begin in the central nervous system, including the brain or the lining around the brain and spinal cord, and sarcomas that begin in an organ or a hollow organ, such as the bowel or urinary tract.

Tumor. Using the TNM system, the "T" plus a number (0 to 2) and letter (a or b) is used to describe the size and location of the tumor. The tumor (T) is further described by its depth. The tumor depth is described as either superficial or deep. Superficial means the tumor is above the body's superficial tissue. Deep means the tumor is either beneath the body's superficial tissue or invades the deep tissue. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no evidence of a primary tumor.

T1: The size of the tumor is 5 centimeters (cm) or smaller.

T1a: The tumor is superficial.

T1b: The tumor is deep.

T2: The size of the tumor is larger than 5 cm.

T2a: The tumor is superficial.

T2b: The tumor is deep.

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

NX: The regional lymph nodes cannot be evaluated.

N0: The cancer has not spread to the regional lymph nodes.

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

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

M0: The cancer has not metastasized.

M1: There has been metastasis to another part of the body.

Grade

Doctors also describe this type of cancer by its grade (G). The grade for sarcoma describes how likely a tumor is to grow and spread. A tumor's grade is described using the letter “G” and a number. There are four grades for sarcoma: GX (the grade cannot be evaluated), G1, G2, and G3. The grade is determined by calculating a score based on the following factors:

  • How different the cancer cells look compared to healthy tissue cells when viewed under a microscope.
  • How many tumor cells are dividing.
  • How much of the tumor has cells that are dying.

The lower the combined score for these three factors, the lower the grade, meaning that the tumor is less aggressive and a patient’s prognosis is better. This is the recommended grading system but others 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.

Stage grouping

Doctors assign the stage of the cancer by combining the T, N, M, and G classifications. For sarcoma, stages I and II are described in smaller groups according to the tumor's depth.

Stage IA: The tumor is 5 cm or smaller in size and either superficial or deep. It has not spread to the lymph nodes or to other parts of the body (T1a or T1b, N0, M0, GX or G1).

Stage IB: The tumor is larger than 5 cm in size and either superficial or deep. It has not spread to the lymph nodes or to other parts of the body (T2a or T2b, N0, M0, GX or G1).

Stage IIA: The tumor is 5 cm or smaller in size and either superficial or deep. It has not spread to the lymph nodes or to other parts of the body. The grade is higher than the grade for stage IA (T1a or T1b, N0, M0, G2 or G3).

Stage IIB: The tumor is larger than 5 cm in size and either superficial or deep. It has not spread to the lymph nodes or to other parts of the body. The grade is higher than the grade for stage IB (T2a or T2b, N0, M0, G2).

Stage III: This stage can be described by either of the following:

  • The tumor is larger than 5 cm in size and either superficial or deep. It has not spread to the lymph nodes or to other parts of the body. The grade is higher than the grade for stage IIB (T2a or T2b, N0, M0, G3).
  • The tumor is any size, either superficial or deep, or any grade. However, it has spread to the regional lymph nodes but not to other parts of the body (any T, N1, M0, any G).

Stage IV: The tumor is any size, either superficial or deep, any grade, and may or may not have spread to the regional lymph nodes. However, it has spread to other parts of the body (any T, any N, M1, and any G).

Recurrent: Recurrent sarcoma is sarcoma that has come back after treatment. If there is a recurrence, the sarcoma may need to be staged again (called re-staging) using the system above.

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Seventh Edition (2010) published by Springer-Verlag New York, www.cancerstaging.net.

Information about the tumor’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for sarcoma. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will learn about the different ways doctors use to treat people with these types of sarcoma. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for sarcomas of specific organs. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new approach to treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Your doctor can help you review all treatment options. For more information, see the 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.

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, since there are so many different types of sarcoma, it is not possible to describe the best treatments for each of the rare sarcomas in this section. It is strongly suggested that people who are diagnosed with a rare type of sarcoma be seen at sarcoma expert centers, since 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. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding 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 specific organs. If the tumor is in an arm or leg, limb-sparing surgical techniques can often be used. 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 of a limb or all of a limb, called amputation. If amputation is needed, rehabilitation that includes physical therapy can help maximize a patient’s physical functioning. It can also help a person cope with the social and emotional effects of losing a limb. Learn more about 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, as well as other parts of the body. As a result, surgery, even liver transplantation, cannot completely eliminate the cancer. Similarly, for 80% of people with cardiac sarcoma, by the time the 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 (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 radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping 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 is delivered through 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 usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Learn more about 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

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 therapy for sarcomas other than liposarcoma or GIST that are not responding to other therapy. It 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.

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

Organ transplantation

Organ transplantation involves using a healthy organ from a donor to replace an organ affected by sarcoma. 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 until a donated organ becomes available. Therefore, patients and their doctors should carefully consider and talk about this treatment option.

Getting care for symptoms and side effects

Sarcoma and its treatment often cause side effects. In addition to treatment 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 can help a person at any stage of illness. People often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both 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, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the sarcoma, such as chemotherapy, surgery, and 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 supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care.

Metastatic sarcoma

If sarcoma has spread to another location in the body, it is called metastatic sarcoma. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of sarcoma because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that might include a combination of chemotherapy, radiation therapy, and possibly surgery. Supportive 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 may be temporary or permanent. This uncertainty leads many survivors to feel worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the sarcoma returning. Understanding the 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 cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies 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 also suggest clinical trials that are studying new ways to treat this type of recurrent sarcoma.

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 fails

Recovery from sarcoma is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and this is difficult to discuss for many people. 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 six 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 think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative 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 helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

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. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat patients with soft tissue sarcomas that are unique to particular organs. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. 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 are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

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 the only way to make 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.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. 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.

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must 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.

For specific topics being studied for sarcoma, learn more in the Latest Research section.

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. 

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.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about sarcoma and how to treat them. To see other pages, use the menu on the side of your screen.

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 diagnostic and treatment options best for you.

Targeted therapy. As described in the Treatment Options section, 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 one new targeted drug, pazopanib, and clinical trials of other drugs that work in different ways to starve tumors of their blood supply are underway.

Immunotherapy. Immunotherapy, also called biologic therapy, is designed to boost the body’s existing defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Immunotherapy is 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 the sarcoma that have never been tested before, providing hope that these new approaches can succeed where previous drugs have failed.

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

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now. Please note this link will take you outside of this guide.

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for sarcoma are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the tumor’s stage, the length and dosage of treatment(s), and your overall health.

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with sarcoma. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your cancer care

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing sarcoma treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will read about your medical care after treatment for sarcoma is finished and why this follow-up care is important. To see other pages in this guide, use the menu on the side of your screen.

After treatment ends for sarcoma, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years, such as a yearly chest x-ray, CT scan, and blood tests.

ASCO offers treatment summary forms to help keep track of the sarcoma treatment you received and develop a survivorship care plan once treatment is completed.

Patients who undergo surgery for sarcoma located in an arm or leg, particularly amputation, often need physical therapy and other types of rehabilitative therapies. Follow-up care should also address the patient’s quality of life, including social and emotional concerns, especially if amputation was necessary. Learn more about cancer rehabilitation.

People recovering from sarcoma are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.

Sarcomas of Specific Organs - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

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. To see other pages, use the menu on the side of your screen.

Talking often with the doctor 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 an e-list and other interactive tools to manage your care.

  • What type of sarcoma do I have?
  • Where exactly is the tumor located?
  • What stage and grade is the sarcoma? What does this mean?
  • Can you explain my pathology report (laboratory test results) to me?
  • How experienced are you in treating this specific type of sarcoma?
  • What are my treatment options?
  • What clinical trials are open to me? Where are they located, and how do I find out more about them?
  • What treatment plan do you recommend? Why?
  • Is surgery an option to remove the sarcoma?
  • Do you recommend any additional treatment?
  • What is the goal of each treatment? Is it to eliminate the sarcoma, help me feel better, or both?
  • Who will be part of my health care team, and what does each member do?
  • Who will be coordinating my overall treatment and follow-up care?
  • What are the possible side effects of this treatment, both in the short term and the long term?
  • 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?
  • How can I keep myself as healthy as possible during and after treatment?
  • Will you recommend specific cancer rehabilitation services for me after treatment?
  • What is my prognosis?
  • What are the chances that the cancer will recur (come back)?
  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?
  • What follow-up tests will I need, and how often will I need them?
  • What support services are available to me? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.  

Sarcomas of Specific Organs - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2014

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. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both 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. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to Sarcomas of Specific Organs. Use the menu on the side of your screen to select another section to continue reading this guide.