Say the word “tumor” and one of the last places you probably think of it occurring is in the heart. Although rare, cardiac tumors occur in approximately 1 in every 2,000 people. Tumors can and do originate in the heart or heart valves. When cardiologists talk about risk factors for these tumors, there just isn’t much to discuss. Although family history may play a role in a very few patients, or it could be tied to some conditions called “syndromes,” most cardiac tumors have no apparent cause.

A tumor that develops in the heart and doesn’t go anywhere else is a primary tumor. One that metastasizes, or begins in another part of the body and eventually makes its way to the heart, is a secondary tumor.    

Recent research finds that primary cardiac tumors represent less than 1 percent of all cardiac tumors, while a tumor that metastasizes from another primary cancer is nearly 30 times more common. Additionally, only about 20 percent of primary cardiac tumors are malignant.

Doctors usually discover cardiac tumors because of a patient’s previously-reported symptoms, while they’re looking for another cardiac-related cause. They utilize advanced cardiovascular imaging techniques that may include:

Computed tomography angiography(CTA)
Echocardiogram
Magnetic resonance imaging (MRI)
Radionuclide imaging

Imaging has double benefits, first enabling identification of the cardiac tumor, then showing the way to treat it. 

Surgery is most frequently the chosen treatment for a cardiac tumor. There are options to perform the surgery as robotically assisted surgery or minimally invasive surgery. Otherwise, doctors may perform more traditional open heart surgery. They worry that a part of the tumor could “break off” and travel somewhere in the circulatory system, causing major problems—called “embolization.”

The most common benign or noncancerous tumors are usually myxomas that occur more frequently in women and grow in the heart’s left atrium, says Eric Roselli, M.D., staff surgeon in the Cleveland Clinic Department of Thoracic and Cardiovascular Surgery. Another common cardiac tumor is the papillary fibroelastoma that usually involves the heart’s valves. Minimally invasive surgery is often effective for these two types. That is, unless they’re so large and vascular in nature—formed from blood vessels—that they require removing the heart from the chest, followed by reconstruction. That’s obviously a bigger, more complex procedure.

“As you might expect, malignant tumors like sarcomas are also more complex to treat, and we may need to use different modalities or types of therapy,” says Dr. Roselli. “Because of our vast multi-disciplinary approach at Cleveland Clinic, a patient’s team here may include cardiologists, cardiovascular imaging specialists, oncologists, radiation oncologists and surgeons.”

Most experts agree that if you’re diagnosed with a cardiac tumor, you owe it to yourself to seek treatment from a cardiac surgeon and a hospital with a strong track record, including outcomes, in this very specialized genre of healthcare.