Heart Conditions in Adults - Cardiac Sarcoma

What is cardiac sarcoma?

Cardiac sarcoma is a type of tumor that occurs in the heart. Cardiac sarcoma is a primary malignant (cancerous)tumor. Tumors are considered to be either primary tumors or secondary tumors. A primary tumor is the original site of tumor growth. A secondary tumor originates from another tumor elsewhere in the body. Primary tumors of the heart are rare, and over 75 percent of primary cardiac tumors are benign (non-cancerous).

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. Angiosarcomas usually begin in the right atrium (right upper chamber) of the heart, or on the pericardium (outer surface) of the heart. Approximately 80 percent of 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 can cause increased fluid in the pericardial sac, the thin covering that surrounds the heart. 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 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 physician for a diagnosis.

How is cardiac sarcoma diagnosed?

The methods for diagnosing cardiac sarcoma vary, to some degree, 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 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 heart. Echocardiography has become the most useful tool in the diagnosis of cardiac sarcoma, allowing the physician to see the exact size and location of the tumor.
• 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 cross-sectional images (often called slices), both horizontally and vertically, 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 which 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 is injected into an artery - to locate the narrowing, occlusions, and other abnormalities of specific arteries.

Treatment for cardiac sarcoma:

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

• your age, overall health, and medical history
• extent 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 (in 80 percent of cases), 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 upon the location and size of the tumor, as well as the extent of metastasis.

The physician 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 which 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. 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.

Click here to view the
Online Resources of Heart Center