Cardiac sarcoma is a rare type of primary malignant (cancerous) tumor that occurs in the heart. 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).

Cardiac Sarcoma Symptoms

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 Angiosarcoma Symptoms

Cardiac sarcomas are most frequently diagnosed as 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. These symptoms occur 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 the following:

• Hemoptysis (coughing up blood)

• Heart rhythm problems

• Upper facial congestion

Other Signs of Cardiac Sarcoma

Other signs of cardiac sarcoma not related to the location of the tumor in the heart may include the following:

• Fever

• Weight loss

• Night sweats

• Malaise (fatigue, tiredness or not feeling well)

• Fingers that change color, or turn blue (Raynaud’s phenomenon), when pressure is applied.

• Nail curvature with enlargement of the finger’s soft tissue.

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

Cardiac Sarcoma Diagnosis

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). This noninvasive test uses sound waves to study 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 that covers 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. A transesophageal echo involves using a flexible tube with a transducer at its tip. This tube is guided down the throat and esophagus. Since the esophagus is located directly behind the heart, a transesophageal echo allows for more detailed pictures of the heart.  

• Electrocardiogram (ECG or EKG). This test records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and detects heart muscle damage. While cardiac sarcoma may cause changes in the heart's rhythm, EKG changes may indicate other heart problems. Therefore, other diagnostic tools are needed to make a definitive diagnosis of cardiac sarcoma or any other type of heart tumor.

• Computed tomography (CT or CAT) scan. This diagnostic imaging procedure 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 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). This diagnostic procedure uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body. The images are used to further define the tumor's size, location and other characteristics.

• Chest X-ray. This diagnostic test uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs on film. This test may detect an enlarged heart or pulmonary congestion.

• Cardiac catheterization. With this procedure, X-rays are taken after a contrast agent (dye) is injected into an artery. The images are used to locate the narrowing, occlusions and other abnormalities of specific arteries.

• Biopsy. This test involves 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 who specializes in evaluating cells, tissues and organs to diagnose disease.

• Blood tests.

Cardiac Sarcoma Treatment

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

• Your age, overall health and medical history

• The extent and location of the disease

• Your tolerance to specific medications, procedures or therapies

• The 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 metastasized (spread) to other parts of the body, making treatment challenging. The type of treatment for cardiac sarcoma depends largely on the location and size of the tumor as well as the extent of metastasis.

Open-Heart Surgery

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

Heart Transplantation

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 (drugs that help prevent the body from rejecting foreign tissue) after the transplant. This medication may stimulate new growth of sarcoma.

Autotransplantation

An exciting new prospect for the treatment of cardiac sarcoma is autotransplantation. During this surgical procedure, 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 returned to the patient’s body. 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, which may be available through clinical trials.

In some cases, such as when the cancer has spread to other parts of the body, radiation therapy or chemotherapy may be given to help relieve symptoms or to improve a person's quality of life.

Causes of Cardiac Sarcoma

Causes of tumors vary, but generally are thought to be the result of abnormal regulation of cell division. Immune system irregularities that are incapable of detecting and fighting aberrant growths can also lead to tumors. Radiation, certain viruses, extreme exposure to the sun, tobacco, poisonous mushrooms and benzene can also cause tumors.

Prevention of Cardiac Sarcoma

By avoiding things that cause cancer, the risk of tumors can be greatly reduced, if not prevented. Don’t smoke or use tobacco products. Reduce, limit or otherwise moderate heavy drinking, excessive exposure to the sun and radiation.

Cancer screenings can detect tumors at early, more treatable stages. Those tests may include mammograms for breast cancer, PAP smears for cervical cancer and colonoscopy for colon cancer.

The earlier a tumor is detected, the more likely a successful treatment outcome. Family members of patients diagnosed with cardiac tumors are advised to undergo screening, since it is inherited.