Cardiac Sarcoma

What is cardiac sarcoma?

Cardiac sarcoma is a rare type of tumor of the heart. Tumors are either primary tumors or secondary tumors. A primary cardiac tumor is one that starts in the heart. A secondary cardiac tumor starts somewhere else in the body and then spreads to the heart. In general, primary tumors of the heart are rare. Most are not cancer (benign), but cardiac sarcoma is cancer.

What are the symptoms of cardiac sarcoma?

The symptoms of heart tumors will vary, depending on where the tumor is. Tumors of the heart may be on the outside surface of the heart, inside one or more chambers of the heart (intracavitary), or 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. The tumor blocks blood flow in and out of the chamber. This blockage may cause symptoms such as swelling of the feet, legs, ankles, or 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 bloodstream 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 the organ or body part. The tiny pieces can affect the brain by causing a stroke. Or they can affect the lungs by causing respiratory distress.

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

The symptoms of cardiac sarcoma may look like other health conditions. Always see your healthcare provider for a diagnosis.

How is cardiac sarcoma diagnosed?

Your healthcare provider will look at your health history and do a physical exam. You will need tests. These may include:

• Echocardiogram (echo). This test that uses sound waves 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. This is because it lets the healthcare provider see the exact size of the tumor and where it is. Transesophageal echo is a type of echo that involves using a flexible tube with a transducer at its tip. The provider guides the tube down your throat and esophagus. The provider can get more detailed pictures of the heart because the esophagus is directly behind the heart.  

• Electrocardiogram (ECG). This test records the electrical activity of the heart. It can show rhythm problems (arrhythmias or dysrhythmias). A cardiac sarcoma may cause changes in the heart's rhythm. The test can also find heart muscle damage. You may need other tests to confirm the diagnosis of cardiac sarcoma or any other type of heart tumor.

• CT scan. This imaging test uses X-rays and computer technology to make detailed images of the body. These include bones, muscles, fat, and organs. CT scans can help your provider better see the tumor's size, location, and other characteristics.

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

• Chest X-ray. This test makes images of internal tissues, bones, and organs. It may show an enlarged heart or pulmonary congestion.

• Cardiac catheterization (angiogram). With this test, the provider takes X-rays after injecting a contrast dye into one of your arteries. This test can help your provider find narrowing, occlusions, and other problems of certain arteries.

• Biopsy. This test removes a small amount of tissue to be looked at under a microscope. Other tests can suggest that the tumor is cancer, but the only way to know for sure is with a biopsy.

Treatment for cardiac sarcoma

Finding out the best treatment for cardiac sarcoma has been difficult because these cancers are so rare. Your doctor will figure out the specific treatment for cardiac sarcoma based on:

• How old you are

• Your overall health and past health

• How sick you are

• How well you can handle specific medicine, procedures, or therapies

• How long the condition is expected to last

• Your opinion or preference

By the time a cardiac sarcoma starts to cause symptoms, it has often spread to other parts of the body (metastasis). This makes treatment difficult and challenging. 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 doctor may see that the tumor can be removed. This will be done with open-heart surgery. The removal is often difficult, because of where the tumor is. You may need chemotherapy after surgery to try to lower the risk that the cancer will come back. It’s not clear how helpful chemotherapy is for this cancer.

In some cases, it may be impossible to remove the sarcoma completely. In this case, your doctor may recommend a heart transplant. You will need to take medicine to help prevent your body from rejecting the transplant. But this medicine may make the sarcoma come back.

Another treatment option is auto-transplantation. In auto-transplantation, the surgeon removes your heart so the tumor can be more easily removed. You will be on a heart-lung bypass machine during the surgery. When the tumor has been removed, the surgeon places the heart back in your chest. Because you don’t have a donor heart, you will not need to take the special medicines to prevent rejection.

If the cancer has spread to other parts of your body, you may get radiation therapy or chemotherapy to help relieve symptoms or improve your quality of life.