Cardiac Sarcoma

Conditions We Treat

What is cardiac sarcoma?

Cardiac sarcoma is a rare type of tumor that occurs in the heart. Cardiac sarcoma is a primary malignant (cancerous) cardiac tumor. Tumors are considered to be 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, and most are benign (noncancerous).

What are the symptoms of cardiac sarcoma?

The symptoms of heart tumors will vary, depending on the location of the tumor. Tumors of the heart may occur on the outside surface of the heart, within one or more chambers of the heart (intracavitary), or within the muscle tissue of the heart.

Cardiac sarcomas, most frequently, are a type of sarcoma called angiosarcoma. Most angiosarcomas occur in the right atrium resulting in obstruction of the inflow or outflow of blood. This obstruction may cause symptoms such as swelling of the feet, legs, ankles, and/or abdomen, and distension of the neck veins, because the blood coming back to the heart after traveling through the body cannot easily enter or be pumped out of the right atrium.

Cardiac angiosarcomas that occur on the pericardium (the thin sac that surrounds the heart) can cause increased fluid inside the sac. If enough fluid accumulates within the pericardial sac, the heart's ability to pump blood is affected. Some signs of this occurrence may include chest pain, shortness of breath, fatigue, and palpitations.

Tiny pieces (emboli) of cardiac sarcomas may break off and travel through the bloodstream to other parts of the body. An embolus may block blood flow to an organ or body part, causing pain and damage to the organ or body part that lies beyond the point at which the blood flow is obstructed. Emboli can affect the brain (causing a stroke), the lungs (causing respiratory distress), and/or other organs and body parts.

Additional symptoms can include hemoptysis (coughing up blood), heart rhythm problems, and upper facial congestion. Other signs of cardiac sarcoma not related to the location of the tumor in the heart may include fever, weight loss, night sweats, and malaise (fatigue, tiredness, or "not feeling well").

The symptoms of cardiac sarcoma may resemble other cardiac or medical conditions. Always consult your doctor for a diagnosis.

How is cardiac sarcoma diagnosed?

The methods for diagnosing cardiac sarcoma vary, to some degree, based on the symptoms present. In addition to a complete medical history and physical examination, diagnostic procedures for cardiac sarcoma may include the following:

• Echocardiogram (also known as an echo). A noninvasive test that uses sound waves to produce a study of the motion of the heart's chambers and valves. The echo sound waves create an image on the monitor as an ultrasound transducer is passed over the skin over the heart. Echocardiography has become the most useful tool in the diagnosis of cardiac sarcoma, allowing the doctor to see the exact size and location of the tumor. Transesophageal echo is a type of echo that involves using a flexible tube with a transducer at its tip. This tube is guided down the throat and esophagus. This allows for more detailed pictures of the heart since the esophagus is directly behind the heart.
• Electrocardiogram (ECG or EKG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage; cardiac sarcoma may cause changes in the heart's rhythm, however, these EKG changes may indicate other heart problems, so other diagnostic tools are needed to make a definitive diagnosis of cardiac sarcoma or any other type of heart tumor.
• Computed tomography (also called a CT or CAT scan). A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays and are used to further define the tumor's size, location, and other characteristics.
• Magnetic resonance imaging (MRI). A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body; to further define the tumor's size, location, and other characteristics.
• Chest X-ray. A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film; may detect heart enlargement or pulmonary congestion.
• Cardiac catheterization. With this procedure, X-rays are taken after a contrast agent (dye) is injected into an artery to locate the narrowing, occlusions, and other abnormalities of specific arteries.
• Biopsy. The removal of a small amount of tissue for examination under a microscope. Other tests can suggest that the tumor is cancerous, but the only way to know for sure is by doing a biopsy. The piece of tumor removed is analyzed by a pathologist (a doctor who specializes in evaluating cells, tissues, and organs to diagnose disease).

Treatment for cardiac sarcoma

Specific treatment for cardiac sarcoma will be determined by the doctor based on:

• Your age, overall health, and medical history
• Extent and location of the disease
• Your tolerance for specific medications, procedures, or therapies
• Expectations for the course of the disease
• Your opinion or preference

Once a cardiac sarcoma has progressed to the point that symptoms begin to occur, it has often spread to other parts of the body, making treatment difficult and challenging. This spread to other parts of the body is called metastasis. The type of treatment for cardiac sarcoma depends largely on the location and size of the tumor, as well as the extent of metastasis.

The doctor may determine that the tumor can be removed, which is done with an open-heart surgical procedure. This is often difficult, however, because of the location of the tumor.

In some cases, the sarcoma has invaded the heart to such an extent that it is impossible to remove it completely. In this situation, heart transplantation has been attempted. However, a patient must receive immunosuppressive medication (medications that help to prevent the body from rejecting foreign tissue) after transplant, and this medication may stimulate new growth of sarcoma.

An exciting new prospect for treatment of cardiac sarcoma is autotransplantation. Autotransplantation is a surgical procedure in which the patient's own heart is removed so that the tumor can be more completely and easily removed from the heart tissue. The patient is placed on a heart-lung bypass machine during the surgery. After the tumor is removed, the heart is replaced into the patient. Because the patient has not received a heart from another person, there is no need for immunosuppressive medications. New drug treatments are also being studied, and may be available in clinical trials.