Cardiac Angiosarcoma

What is cardiac angiosarcoma?

Cardiac angiosarcoma is a malignant (cancerous) tumor in your heart. It starts in the lining of your heart’s blood vessels and spreads to your heart muscle.

In 80% of cases, this rare tumor happens in your right atrium (upper heart chamber). It can also show up in your right ventricle (lower heart chamber) or pericardium (the protective sac around the outside of your heart). Rarely, it can be in the left side of your heart.

This aggressive, invasive tumor can block or push against areas (like blood vessels and valves) around it. The invasiveness of this tumor can make it hard to remove with surgery.

How common is cardiac angiosarcoma?

Autopsy results have shown that fewer than .03% of people had a primary cardiac neoplasm (abnormal tissue growth). As part of that category, cardiac angiosarcoma is rare. But it’s the most common kind of malignant tumor an adult can get in their heart.

People usually get a diagnosis before age 65, usually between their 20s and 40s. These tumors happen two to three times more often in people assigned male at birth (AMAB).

What are the symptoms?

Cardiac angiosarcoma symptoms vary depending on the location of the tumor.

Symptoms may include:

• Shortness of breath (dyspnea).
• Chest pain.
• Fatigue.
• Syncope (passing out).
• Heart palpitations.

If the tumor spreads beyond your heart, you may have other symptoms, like anemia or weight loss.

What causes cardiac angiosarcoma (angiosarcoma of the heart)?

There isn’t a clear cardiac angiosarcoma cause. But people with certain mutations (changes) in their DNA, like the POT1 gene, may be more likely to get cardiac angiosarcoma. Another possibility is that people who get this disease received radiation therapy to their chest to treat childhood cancer.

What are the risk factors?

Risk factors for cardiac angiosarcoma include:

1. A history of radiation therapy for treatment of another cancer.
2. Exposure to certain chemicals that can cause cancer.
3. Lymphedema.

What are the complications of cardiac angiosarcoma?

Complications of cardiac angiosarcoma may include:

1. Heart failure.
2. Blockage in your vena cava (large blood vessel).
3. Arrhythmia (abnormal heart rhythm).
4. Pericardial effusion.
5. Cardiac tamponade.
6. Heart valve regurgitation (leak).
7. Obstructive shock.

How is cardiac angiosarcoma diagnosed?

A healthcare provider can make a cardiac angiosarcoma diagnosis by using:

• Transthoracic echocardiogram (noninvasive).
• Transesophageal echocardiogram (invasive).
• Computed tomography (CT).
• Heart MRI (magnetic resonance imaging).
• Positron emission tomography (PET).
• Cardiac catheterization.
• Heart biopsy.

How is cardiac angiosarcoma treated?

Surgery can improve your symptoms. Removing the whole tumor gives you the best chance of surviving. But it can be difficult to remove the entire tumor if it’s spread to other areas of your heart. Choosing a surgeon who has experience with this kind of rare tumor may increase your chances of a complete tumor removal.

Chemotherapy can ease your symptoms. It can also help some people live longer. Sometimes, radiation can improve your situation.

Complications of the treatment

Complications of surgery may include:

• Bleeding.
• Abnormal heart rhythm (arrhythmia).
• Heart dysfunction.

How can I lower my risk of cardiac angiosarcoma?

You may be able to lower your risk of cardiac angiosarcoma by limiting your exposure to certain chemicals. If you have a genetic irregularity that puts you at risk for this kind of cancer, talk to your provider. You may be able to get screenings that could catch cardiac angiosarcoma early.

What can I expect if I have cardiac angiosarcoma?

A tumor can return after treatment. It’s common for cardiac angiosarcoma to spread to your liver, lungs and brain before you get a diagnosis. It can also spread to your bones and adrenal glands. This is what makes it a difficult cancer to treat and survive.

Outlook for cardiac angiosarcoma

The cardiac angiosarcoma prognosis is very poor. The disease aggressively invades the areas around it. The average survival time for a person with a cardiac sarcoma is about 10 to 16 months. Without surgery, most people live 9 to 12 months (even if they’ve tried other kinds of treatment).

Other estimates say survival time can be as short as 5 months or less.

About 14% of people with a cardiac angiosarcoma diagnosis are alive five years later. Cardiac angiosarcoma that spreads (metastasizes) to other body parts is often fatal.

How do I take care of myself?

The basic things you do when you’re healthy are still important when you’re fighting cancer. Eating nutritious foods ensures your body’s getting the nutrition it needs. Getting enough rest is even more important if treatments make you tired.

If you need to relax, try yoga, meditation or deep breathing. You may also want to find a support group (even if it’s online) where you can safely share what you’re feeling.

When you’re nearing the end of your life, hospice care can help keep you comfortable.

When should I see my healthcare provider?

Keep all scheduled appointments with your provider. But don’t hesitate to contact them if:

• Your symptoms come back.
• Your symptoms get worse.
• You need help with side effects from treatment.

When should I go to the ER?

Get emergency help if you have:

• Shortness of breath.
• Chest pain.
• A fever or other signs of infection if you’ve had chemotherapy.

What questions should I ask my doctor?

Since cardiac angiosarcoma is rare, it may be hard to find much information about it. You may want to ask your healthcare provider:

• Has this cancer spread to other parts of my body?
• Is surgery an option for me?
• From looking at imaging, does it seem likely that you can remove the whole tumor?
• Which treatments do you recommend for me?
• What’s the outlook for someone in my situation?

A note from Cleveland Clinic

Most people have never heard of cardiac angiosarcoma. It can be difficult to explain your situation to your family and friends. Don’t be afraid to ask your provider about anything you don’t understand. Take advantage of any resources or services available to help you. If you don’t have a good prognosis, talk to someone you trust about what you want. It may give you comfort to talk to a counselor and arrange hospice services.