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Sarcoma - Alveolar Soft Part and Cardiac - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 Alveolar Soft Part Sarcoma and Cardiac Sarcoma. 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 benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

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 alveolar soft part sarcoma and cardiac sarcoma

Because there are several different types of soft tissue sarcoma, it is considered a family of related diseases, rather than a single, specific disease. Two specific types of sarcoma, alveolar soft part sarcoma (ASPS) and cardiac sarcoma, are discussed in this section. You can learn more about other types of sarcoma elsewhere on Cancer.Net.

ASPS is a slow-growing tumor that usually begins in the thigh or leg, but it sometimes develops on the head or neck. People with ASPS have a specific genetic change called a translocation. See the Risk Factors section for more details.

Cardiac sarcoma is a rare tumor that occurs in or on the heart. Although most heart tumors are benign, a cardiac sarcoma is usually cancerous. The most common tumor type is an angiosarcoma. This tumor occurs in one of the chambers of the heart or within the muscle tissue of the heart. In fact, about 25% of angiosarcomas develop inside the heart, blocking the flow of blood and causing symptoms.

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.

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Sarcoma - Alveolar Soft Part and Cardiac - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE:You will find information about how many people learn they have 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 12,020 people (6,550 males and 5,470 females) will be diagnosed with a soft tissue sarcoma in the United States. An estimated 4,740 adults and children (2,550 males and 2,190 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, but the actual risk for a particular individual may differ. 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 2014.

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Sarcoma - Alveolar Soft Part and Cardiac - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about what factors increase the chance of developing ASPS. 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.

The following factor can raise a person’s risk of developing ASPS:

Genetics. Research has shown that people with ASPS often have a genetic abnormality or mutation in which part of one chromosome (a long strand of genes) breaks off and reattaches to another chromosome. This is called a translocation. The translocation causes two genes called ASPL and TFE3 to merge into one gene called ASPL-TFE3. This mutation is found only in the tumor cells, not in other organs of the body, and is not inherited (passed down from one generation to the next). Therefore, family members of a person diagnosed with ASPS do not have an increased risk of developing ASPS themselves. The ASPL-TFE3 gene causes cells to make an abnormal enzyme that causes soft tissue cells to grow out of control.

There are no known risk factors for cardiac sarcoma.

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Sarcoma - Alveolar Soft Part and Cardiac - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 ASPS or cardiac sarcoma may experience the following symptoms or signs. Sometimes, people with these types of tumors do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Symptoms of ASPS may include:

  • A painless mass (growth) in the leg or 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 not being able to move the limb (leg or arm) well

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 to produce these symptoms:

  • Swelling of the feet, legs, ankles, or abdomen
  • Distention (stretching) of neck veins (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 (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
  • Heart palpitations (forced or irregular heart beat)

Because the heart pumps blood into the rest of the body, emboli (fragments of the tumor) may break away from the main tumor and travel throughout the body. The fragments can lodge in other organs or block blood flow to an organ or other body part. If this happens, symptoms depend on which organ(s) is blocked and may include:

  • Difficulty breathing (if the tumor fragment is in the lungs)
  • Stroke (if the brain is affected)
  • Damage to other organs and body parts

Other symptoms of cardiac sarcoma may include:

  • Coughing up blood
  • Fever
  • Unexplained weight loss
  • Night sweats
  • Fatigue
  • Feeling unwell
  • Heart rhythm problems
  • Upper body congestion

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If sarcoma is diagnosed, relieving symptoms and side effects 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.

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Sarcoma - Alveolar Soft Part and Cardiac - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 a sarcoma and find out if it has metastasized (spread). 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 ASPS or cardiac sarcoma:

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 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 contrast medium (a special dye) is injected into a patient’s vein or given orally (by mouth) to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein or given orally to create a clearer picture.

The following tests may also be used to diagnose ASPS:

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.

In addition, the following tests may be used to diagnose cardiac sarcoma:

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.

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.

After these 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.

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.

Sarcoma - Alveolar Soft Part and Cardiac - Stages and Grades

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. 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 determine 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 (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.

Alveolar soft part sarcoma and cardiac sarcoma are included in the staging of soft tissue sarcoma, described below.

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 (the tumor is above the body's superficial tissue) or deep (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 (on the surface or shallow).

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 (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).

This staging system applies to all types of soft tissue sarcoma except Kaposi sarcoma, 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.

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

Sarcoma - Alveolar Soft Part and Cardiac - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 ASPS and cardiac sarcoma. 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 treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. 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 ASPS and cardiac sarcoma are listed below. Treatment options and recommendations depend on several factors, including the type and stage of the sarcoma, possible side effects, and the patient’s preferences and overall health. Take time to learn about 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 ASPS. 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.

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 may be used (see below).

Learn more about cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill 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 kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. 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.

Unfortunately, chemotherapy is not very effective for ASPS or cardiac sarcoma and often causes serious side effects. Therefore, newer drug treatment options, such as cediranib for ASPS (see Targeted Therapy below) or other drugs currently being studied in clinical trials, may be recommended instead.

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.

Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells.

Recent studies have discovered specific genetic mutations (changes) found in ASPS cells that are not found in healthy cells (see the Risk Factors section). These changes cause ASPS genes to be “turned on,” causing cells to grow uncontrollably, spread into other tissues, and eventually metastasize to other parts of the body. Drugs are now being developed that “turn off” or target these genes.

Cediranib (Recentin, AZD2171) is a drug currently being studied in clinical trials that specifically targets three genes that are known to promote the growth of ASPS through angiogenesis (the process of making new blood vessels). Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies is to “starve” the tumor.

In a recently published study, 46 patients who had ASPS that could not be surgically removed were given cediranib every day. Approximately one-third of the patients had significant decrease in the size of the tumor that lasted months or, in some cases, years without the tumor re-growing. This study also suggested that for people whose tumor did not shrink, cediranib still may have kept the tumor from growing for a longer period of time than would have been expected.

The most common side effects of cediranib are diarrhea and high blood pressure. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Many other research studies are now taking place to find out more about cediranib and other targeted therapies for ASPS (see the Latest Research section for more information).

Learn more about targeted treatments.

Heart transplantation

Heart transplantation involves using a heart from a donor to replace the heart of a person with cardiac sarcoma. For a transplant to be successful, the patient will have to take immunosuppressive medication (medications that help a patient’s body accept the new heart). As a result of taking this medication, the patient could develop a new cancer or the sarcoma might come back; 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.

Recurrent ASPS or cardiac sarcoma

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

A remission can 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, including whether the sarcoma’s stage has changed. 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.

Metastatic ASPS or cardiac 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.

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 bereavement.

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.

Sarcoma - Alveolar Soft Part and Cardiac - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 ASPS or cardiac sarcoma. 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 treating ASPS or cardiac sarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with ASPS or cardiac 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 ASPS and cardiac 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.

Sarcoma - Alveolar Soft Part and Cardiac - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will read about the scientific research being done now to learn more about ASPS and cardiac 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 ASPS and cardiac 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 are currently focusing on the use of angiogenesis inhibitors, a type of targeted therapy that is focused on stopping angiogenesis, to treat ASPS. Currently two of these drugs are being tested for people with ASPS: cediranib and sunitinib (Sutent).

Immunotherapy. Immunotherapy (also called biologic therapy) is designed to boost the body’s natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Immunotherapy is being researched as a treatment option for ASPS. Learn more about immunotherapy.

Chemotherapy. New drugs are also being researched for the treatment of ASPS.

Autotransplantation. For cardiac sarcoma, researchers are looking at autotransplantation, a procedure where the person’s own heart is removed from the body during surgery so cardiac sarcoma can be more easily removed from the heart. After completely removing the tumor from the heart, the heart is put back into the patient. There is no need for immunosuppressive drugs because the person’s own heart is used in the procedure.

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.

Sarcoma - Alveolar Soft Part and Cardiac - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 ASPS or cardiac 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 ASPS or cardiac sarcoma. Learn more about caregiving.

In addition to physical side effects, there may be psychosocial (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. Learn more about the importance of addressing such needs, 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 cancer 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.

Sarcoma - Alveolar Soft Part and Cardiac - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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

After treatment ends for ASPS or cardiac 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 of the chest, 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 ASPS of a limb, 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 ASPS or cardiac 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.

Sarcoma - Alveolar Soft Part and Cardiac - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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?
  • What are my treatment options?
  • What clinical trials are open to me?
  • What treatment plan do you recommend? Why?
  • How successful would surgery be to remove it?
  • Do you recommend additional treatments?
  • What is the goal of each treatment? Is it to eliminate the sarcoma, help me feel better, or both?
  • What is my prognosis?
  • 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?
  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?
  • What are the chances that the cancer will recur (come back)?
  • 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 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.

Sarcoma - Alveolar Soft Part and Cardiac - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 Alveolar Soft Part Sarcoma and Cardiac Sarcoma. 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 Alveolar Soft Part Sarcoma and Cardiac Sarcoma. Use the menu on the side of your screen to select another section to continue reading this guide.