Cardiac Sarcoma

What is cardiac sarcoma?

Cancer is made of abnormal cells that grow out of control. The abnormal cells often grow to form a lump or mass called a tumor. Cancer cells can also grow into (invade) nearby areas. And they can spread to other parts of the body. This is called metastasis.

Cardiac sarcoma is a type of cancer that starts in the muscle or blood vessels of the heart. The cancer can be either primary or secondary. A primary cardiac tumor is one that starts in the heart. It is very rare. A secondary cardiac tumor is from cancer that starts somewhere else in the body and then spreads to the heart. Secondary cardiac tumors are more common than primary tumors.

What are the symptoms of cardiac sarcoma?

The symptoms of heart tumors vary, depending on where the tumor is. Tumors of the heart may be on the outside surface of the heart. They may be inside one or more chambers of the heart (intracavitary). Or they may be within the heart muscle tissue.

Cardiac sarcomas are most often a type of sarcoma called angiosarcoma. Most angiosarcomas occur in the right upper chamber (atrium) of the heart, but they can also be found in the left atrium. The tumor blocks blood flow in and out of the chamber. This blockage may cause symptoms like chest pain and swelling of the feet, legs, ankles, or belly (abdomen). It may cause the veins in your neck to stand out (distend). This is because the blood coming back to the heart after traveling through the body can’t easily go into or be pumped out of the right atrium.

Cardiac angiosarcomas that occur on the thin sac that surrounds the heart (pericardium) can cause fluid to collect in the sac. If enough fluid builds up, it affects how well the heart can pump blood. Some signs of this buildup may include chest pain, shortness of breath, fatigue, and fluttering heartbeat (palpitations).

Tiny pieces (emboli) of cardiac sarcomas may break off and travel through the blood to other parts of the body. The tiny pieces may block blood flow to an organ or body part. This can cause pain and damage to that organ or body part. The tiny pieces can affect the brain by causing a stroke. Or they can affect the lungs by causing shortness of breath.

Other symptoms can include coughing up blood (hemoptysis), heart rhythm problems, hoarse voice, and swelling in the face. Signs of cardiac sarcoma not related to where the tumor is in the heart may include fever, weight loss, night sweats, fatigue, and a general feeling of not being well.

The symptoms of cardiac sarcoma often seem like other health conditions. It's important to see a healthcare provider if you have symptoms. Only a healthcare provider can tell if you have cancer or some other problem that needs to be treated.

How is cardiac sarcoma diagnosed?

Your healthcare provider will ask about your symptoms and medical history. They will give you a physical exam. You will need tests to look at your heart and see how well it's working. These may include:

• Echocardiogram (echo). This test uses sound waves (like an ultrasound) to look at how well your heart’s chambers and valves are working. This test has become the most useful tool in diagnosing cardiac sarcoma. It lets the healthcare provider see the exact size of the tumor and where it is. Transesophageal echo is a type of echo that uses a thin, flexible tube with a transducer at its tip. The provider guides the tube down your throat and into your esophagus. This gives more detailed pictures of the heart because the esophagus is right behind the heart.

• Electrocardiogram (ECG). This test records the electrical activity of your heart. It can show rhythm problems. These are also known as arrhythmias or dysrhythmias. Cardiac sarcoma may cause changes in the heart's rhythm. The test can also show signs of heart muscle damage.

• CT scan. This imaging test uses X-rays and a computer to make detailed images of the inside of your body from different angles. These include bones, muscles, fat, and organs. CT scans can help your provider better see how big the tumor is, exactly where it is, and other features.

• MRI. This imaging test uses large magnets, radio waves, and a computer to make detailed images of organs and tissues inside your body. This test also helps your provider better see the tumor's size, location, and other features.

• PET (positron emission tomography) scan. This imaging test may be done to find where the tumor is. For a PET scan, a safe, radioactive liquid (tracer) is injected into the bloodstream. It takes about 45 minutes for the tracer to be in your system. Once the tracer is there, the healthcare provider takes a scan of your body.

• Chest X-ray. This test makes images of internal tissues, bones, and organs. It may show an enlarged heart. Or it may show fluid in your lungs (pulmonary congestion).

• Cardiac catheterization (angiogram). With this test, the provider threads a long flexible tube (catheter) through your blood vessels into your heart. Then they take X-rays after injecting a contrast dye into 1 of your arteries. This test can show narrowing, blockages, and other problems of certain arteries.

• Biopsy. This is done by taking out a small sample of the tumor for testing. Other tests can suggest that the tumor is cancer, but a biopsy is the only way to know for sure.

How is cardiac sarcoma treated?

Finding the best treatment for cardiac sarcoma can be difficult. This is because these cancers are so rare. The treatment plan for cardiac sarcoma is based on:

• Your age

• Your overall health and past health

• How sick you are

• How well you can handle certain medicines, procedures, or therapies

• How fast the cancer cells are growing

• If the tumor can be removed or the cancer has spread

• Your opinion and preferences

By the time a cardiac sarcoma starts to cause symptoms, it has often spread to other parts of the body (metastasized). This makes treatment difficult. The type of treatment for cardiac sarcoma depends mainly on where the tumor is and how big it is. It also depends on how far it has spread.

The provider may see that the tumor can be removed. This is done with open-heart surgery. You may need chemotherapy (chemo) after surgery to try to lower the risk of the cancer coming back. It’s not yet known how well chemo works for this cancer.

In some cases, it may not be possible to remove all of the tumor. In this case, your provider may advise a heart transplant. You'll need to take medicine for the rest of your life. This is to keep your body from rejecting the transplant. But this medicine may increase the risk that the sarcoma will come back (recur).

Another treatment option is autotransplantation. For this, the surgeon takes your heart so the tumor can be more easily removed. You'll be on a heart-lung bypass machine during the surgery. When the tumor has been removed, the surgeon puts your heart back. Because you don’t have a donor heart, you won't need to take medicines to prevent rejection.

If the cancer has spread to other parts of your body, you may get radiation therapy or chemo. This is done to help relieve symptoms, help you live longer, or improve your quality of life.

Talk with your healthcare providers about the risks, benefits, and possible side effects of all treatments.

What are possible complications of cardiac sarcoma?

Cancer treatment such as chemo and radiation can damage normal cells. This causes side effects. Talk with your healthcare provider about ways to manage your side effects. There may be things you can do and medicines you can take to help prevent or control side effects.

Can cardiac sarcoma be prevented?

Researchers don’t yet know how to prevent this type of cancer.

When should I call my healthcare provider?

Call your healthcare provider if you have:

• Symptoms that don’t get better, or get worse

• New symptoms

Key points about cardiac sarcoma

• Cardiac sarcoma is a type of cancer that starts in the muscle or blood vessels of the heart.

• The symptoms of heart tumors vary, depending on where the tumor is. They can include swelling of legs or belly, fatigue, chest pain, and other symptoms.

• You will need tests to look at your heart and see how well it's working. These may include an echocardiogram, ECG, imaging scans, or other tests.

• Finding the best treatment for cardiac sarcoma can be difficult. Treatment may include surgery, radiation therapy, or chemotherapy.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are and when they should be reported.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your provider if you have questions, especially after office hours and on weekends and holidays.