Heart Cancer

Overview

What is heart cancer?

Heart cancer occurs when diseased cells grow out of control on or near the heart. These cells form a tumor. Cancer that begins in the heart is primary heart cancer. This form of heart cancer is extremely rare.

Heart cancer more commonly occurs when cancer cells spread to the heart from cancer in a nearby organ. For example, lung cancer may spread to the heart, causing secondary heart cancer. Cancer that spreads is metastatic cancer.

How common is heart cancer?

Primary heart cancer affects fewer than 2 out of 100,000 people every year. Cardiac tumors are rare. An estimated 8 out of 10 tumors that affect the heart are benign (not cancer).

Why is heart cancer so rare?

The heart is made of connective tissue and muscle cells that do not turn over very fast, which makes them very resistant to becoming cancerous. Cancer cells grow and multiply more vigorously in epithelial tissue that tends to turn over more quickly and so is more susceptible to a mutation (error in replication) occurring that can lead to cancer.

Epithelial tissue lines most organs. The breasts also have this tissue. For this reason, cancer more commonly affects tissue in the breasts and organs like the colon, pancreas, lungs and skin.

Who might get heart cancer?

Anyone can get heart cancer. The disease is more likely to affect men aged 30 to 50, but only slightly more often than in women. People who smoke or have AIDS may be more at risk but the data is limited.

Symptoms and Causes

What causes heart cancer?

Angiosarcoma, a rare type of malignant (cancerous) soft tissue tumor, causes 9 out of 10 primary heart cancers in adults. The cause of angiosarcoma is unknown, although radiation and some toxins may play a role. Other subtypes of sarcoma cancerous tumors can also occur in the heart and great vessels (aorta, pulmonary arteries).

Secondary heart cancer is 30 to 40 times more likely than cancer that originates in the heart. Metastatic cancer can spread to the heart from a diseased organ, including the skin, lungs and kidneys. It can also spread from cancers of the thymus gland which lies in the chest, or from the blood (leukemia) and lymphatic system (lymphoma).

Is heart cancer inherited?

Angiosarcomas of the heart sometimes affect multiple members of the same family. Scientists believe that certain people are more prone to this primary heart cancer because of their genes, but we are still learning about the genetic basis of most of these cancers.

The cause may be related to a mutation (change) in a gene called protection of telomeres protein 1 (POT1). A parent with this gene mutation can pass it to their children.

What are the symptoms of heart cancer?

Sudden unexplained heart failure is a top sign of heart cancer. You may experience shortness of breath and extreme fatigue if the tumor is pressing on a chamber of the heart or growing within the heart and affecting valve function.

Other symptoms of heart cancer include:

Many cardiac tumors are found incidentally on an imaging study like an echocardiogram (heart ultrasound) or CT scan or MRI done for another reason.

Primary heart cancer can spread to other parts of the body. It most commonly spreads to the nervous system, such as the spine or brain. It can also travel to the lungs. Symptoms of heart cancer that spreads include:

Diagnosis and Tests

How is heart cancer diagnosed?

In people with heart cancer, healthcare providers often find the tumor while looking for the cause of heart problems. You may get one or more of these tests:

Management and Treatment

What are the complications of heart cancer?

Heart cancer affects your heart function. It can lead to a host of potentially life-threatening problems. With a heart tumor, you have a higher risk of having a heart attack, stroke or severe heart failure.

Small pieces of a heart tumor can break free and travel through the bloodstream. These pieces can lodge in a blood vessel, causing a blood clot that can lead to stroke if it goes to the brain, or respiratory problems if it goes to the lungs.

Heart cancer can affect the pericardium, the sac that surrounds the heart. Inflammation called pericarditis can result. Your healthcare provider may need to use a catheter (long, thin tube) to drain excess fluid and ease pressure on the heart and send the cells in the fluid for diagnostic analysis. This procedure is pericardiocentesis.

How is heart cancer managed or treated?

Chemotherapy or radiation therapy (or a combination of both) can sometimes shrink a heart tumor and relieve symptoms. If another cancer spread to the heart, your healthcare provider will treat that primary cancer.

Other treatment depends on the tumor location and size, as well as factors like your overall health and age. Surgery to remove the tumor may be an option, and has been shown to prolong life if it can be completely removed. These operations can be quite complex and are best performed at a specialty center. You may also be able to join a clinical trial to try promising new therapies still in development.

Prevention

How can I prevent heart cancer?

Experts don’t fully understand why some people develop this rare cancer. Smoking contributes to many cancers and also leads to heart disease. Your healthcare provider can help you quit smoking.

Outlook / Prognosis

What is the prognosis (outlook) for people who have heart cancer?

There isn’t a cure for heart cancer, and the disease is difficult to treat. After treatment, heart cancer often returns and may spread to other parts of the body.

The average life expectancy after a heart cancer diagnosis is about six months without surgical treatment, and greater than a year when surgery is possible with some reports of patients surviving several years after a complete resection of the tumor. It’s important to remember that researchers continue to look for better ways to detect this disease early, refine current treatments, and find new ones.

Living With

When should I call the doctor about heart cancer concerns?

You should call your healthcare provider if you experience:

  • Changes in heart rate or rhythm.
  • Chest pain or shortness of breath.
  • Chronic back pain.
  • Confusion or memory problems.
  • Coughing up blood.
  • Dizziness or fainting.

What questions should I ask my doctor about heart cancer?

You may want to ask your healthcare provider:

  • What type of heart cancer do I have?
  • What treatments can help?
  • How can I manage treatment side effects?
  • Should I look out for signs of complications?

A note from Cleveland Clinic

Because heart cancer is so rare, healthcare providers may misdiagnose the cause of heart problems. Specific diagnostic tests can help identify a heart tumor. If another cancer spreads to the heart, your provider will continue to treat that cancer. Standard cancer treatments like chemotherapy, radiation therapy and surgery can shrink or remove heart tumors and relieve symptoms. Researchers are exploring better ways to treat this uncommon disease.

Last reviewed by a Cleveland Clinic medical professional on 05/12/2021.

References

  • American College of Cardiology. Accessed 5/17/2021.A Review of Malignant Primary Cardiac Tumors. (https://www.acc.org/latest-in-cardiology/articles/2018/04/20/08/41/a-review-of-malignant-primary-cardiac-tumors)
  • American Heart Association. Accessed 5/17/2021.The Heart Isn’t Particularly Vulnerable to Cancer — And Here’s Why. (https://www.heart.org/en/news/2019/02/25/the-heart-isnt-particularly-vulnerable-to-cancer-and-heres-why)
  • Cresti A, Chiavarelli M, et al. . J Cardiovasc Med. 2016;17(1):37-43. Accessed 5/17/2021.Incidence rate of primary cardiac tumors: A 14-year population study (https://pubmed.ncbi.nlm.nih.gov/25022931/)
  • Hoffmeier A, Sindermann JR, Scheld HH, Martens S. Dtsch Arztebl Int. 2014;111(12):205-11. Accessed 5/17/2021.Cardiac tumors — Diagnosis and surgical treatment. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3983698/)
  • The Liddy Shriver Sarcoma Initiative. Accessed 5/17/2021.What is Angiosarcoma? (http://sarcomahelp.org/angiosarcoma.htm)
  • Merck Manual Consumer Version. Accessed 5/17/2021. Cancerous Heart Tumors. (https://www.merckmanuals.com/home/heart-and-blood-vessel-disorders/heart-tumors/cancerous-heart-tumors)
  • National Cancer Institute. Accessed 5/17/2021.Matters of the Heart: Why Are Cardiac Tumors So Rare? (https://www.cancer.gov/types/metastatic-cancer/research/cardiac-tumors)
  • Shapiro LM. Heart. 2001;85:218-22. Accessed 5/17/2021.Cardiac tumors: Diagnosis and management. (https://heart.bmj.com/content/85/2/218)

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