Papillary Fibroelastoma

What is a papillary fibroelastoma?

A papillary fibroelastoma is a benign (noncancerous) heart tumor. It’s considered a primary heart tumor because it begins in your heart.

Even though it’s not cancerous, a papillary fibroelastoma is dangerous because it can become embolic. That means it can break off and travel somewhere else in your body, causing serious complications such as a stroke.

In 4 out of 5 cases, papillary fibroelastomas grow on your heart valves. In fact, it’s the most common type of tumor that involves your valves.

Which valve is most often affected by papillary fibroelastomas?

Papillary fibroelastomas usually affect your aortic valve (most common) or mitral valve. These are the valves on the left side of your heart. Less often, they affect your tricuspid valve or pulmonary valve on the right side of your heart.

Papillary fibroelastomas usually don’t interfere with your valve function. That’s because they’re typically on the downstream side of your valve, meaning the part where blood flows away from the valve.

This makes papillary fibroelastomas different from cardiac myxomas. Myxomas are larger in size and more likely than papillary fibroelastomas to interfere with valve function. In addition, myxomas are less likely to be attached to the valves unlike papillary fibroelastomas.

Where are papillary fibroelastomas located?

These tumors are usually located on your heart valves. About 95% of the time they’re on the left side of your heart. Rarely do they grow from the lining of your heart chambers, particularly in your left ventricle.

How do papillary fibroelastomas affect my body?

Papillary fibroelastomas can trigger blood clots that travel through your bloodstream. These blood clots can travel to many different parts of your body, including your:

• Brain.
• Coronary arteries.
• Kidneys.
• Lungs.
• Retinas.
• Spinal cord.

These traveling clots, known as emboli, can cause:

• Heart attack.
• Stroke.
• Ventricular fibrillation.

Papillary fibroelastomas can also affect blood flow through your heart. If the tumor attaches to your aortic valve, it can prevent blood from reaching your heart through your coronary arteries. This can cause angina during physical activity and put you at risk for sudden cardiac death.

Who is affected by papillary fibroelastomas?

Papillary fibroelastomas usually affect adults over age 40. Most people are diagnosed around age 60. Rarely, these tumors affect babies and children who have congenital heart defects.

How common are papillary fibroelastomas?

Primary heart tumors affect fewer than 1 in 2,000 people. Papillary fibroelastomas and cardiac myxomas are the most common noncancerous primary heart tumors. Scientists don’t know the exact prevalence since many aren’t found until after the person dies (post-mortem).

What are the symptoms of a papillary fibroelastoma?

More than half of people don’t have any symptoms. Those who do have symptoms may experience:

• Angina.
• Syncope (fainting).
• Shortness of breath.
• Transient ischemic attack or stroke.

What causes a papillary fibroelastoma?

The exact cause of papillary fibroelastomas isn’t known. However, many scientists believe they’re caused by damage to your endothelial cells, which form the lining of your heart. Small blood clots join together and move to the area that’s damaged, ultimately forming a growth.

How are papillary fibroelastomas diagnosed?

Papillary fibroelastomas are usually diagnosed incidentally. That means your provider finds a tumor through imaging tests run for another reason. Providers also diagnose these tumors when looking for the cause of a stroke.

Tests to diagnose a papillary fibroelastoma

Echocardiography is the most common method for diagnosing papillary fibroelastomas. Providers usually run a transthoracic echo first. But in some cases, they also need to run a transesophageal echo to check for very small tumors or see the affected area in greater detail.

What does a papillary fibroelastoma look like on an echo?

A papillary fibroelastoma looks like a sea anemone on an echocardiogram. That’s because it has papillary fronds that extend from its surface.

The tumor usually appears small (less than 1 centimeter). It’s typically pedunculated, which means it’s attached to a stalk (stem). This stalk allows the tumor to be very mobile and sway back and forth.

What is the treatment for papillary fibroelastomas?

Surgery is the typical treatment for papillary fibroelastomas. Your surgeon will:

• Remove the entire tumor.
• Check the rest of your heart to make sure there are no other tumors.
• Fix any parts of your heart that the tumor damaged. This may involve valve repair or replacement.

Your provider may recommend surgery even if you don’t have symptoms. This is to reduce your risk of future complications from the tumor.

Surgery usually involves traditional open-heart methods. But some people are candidates for minimally invasive robotic methods. Talk with your provider about the method that’s best for you.

If surgery is too risky for you, then your provider may recommend anticoagulants (blood-thinners) to lower your risk of blood clots.

What is the prognosis for people with papillary fibroelastomas?

People who have surgery to remove a papillary fibroelastoma have a good prognosis. There’s a low risk of the tumor coming back. Since it’s not cancerous, there’s no risk of it spreading.

When should I see my healthcare provider?

Since cardiac tumors are often diagnosed by accident, it’s important to keep all your medical appointments and follow-ups. Tumors diagnosed early are easier to treat. Left untreated, papillary fibroelastomas can get bigger and lead to serious complications.

If you have open-heart surgery to remove a tumor, your body will need time to recover. Talk with your provider about activity restrictions in the weeks following your surgery. Your provider may recommend cardiac rehab to help you gain back your strength.

What questions should I ask my provider?

If you’ve been diagnosed with a papillary fibroelastoma, ask your provider:

• How big is the tumor?
• Where is it located?
• How is it affecting my heart?
• Will I need surgery? If so, how soon?
• How can I prepare for the surgery?
• What’s the recovery like?
• What are the risks of surgery?
• What’s my outlook following treatment?
• How often should I come back for follow-ups or additional tests?

What does a mass on your heart mean?

A mass on your heart is any type of abnormal growth (tumor) located in your heart. Doctors call these growths “cardiac tumors” or “heart tumors.” Heart tumors are either cancerous or noncancerous. They may begin in your heart (primary) or spread to your heart from somewhere else in your body (metastatic).

It’s rare for a tumor to begin in your heart. But among those that do, papillary fibroelastomas are one of the most common forms, along with cardiac myxomas.

A note from Cleveland Clinic

Learning you have a heart tumor is frightening. But the good news is that a papillary fibroelastoma is benign and isn’t cancerous, meaning it won’t spread anywhere else in your body. And it usually can be cured through surgery. Talk with your provider about the best treatment plan for you and what you can expect going forward.