Cardiac angiosarcoma is a rare form of cancer that occurs in your cardiac (heart) tissue. An angiosarcoma is a type of cancer in which cancerous cells grow in your lymph or in the inner lining of your blood vessels. While cardiac angiosarcoma is rare, it’s the most common cause of malignant (cancerous) tumors in the heart.

A cardiac angiosarcoma can obstruct blood flow in your heart. It can also affect your heart’s ability to pump blood.

Cardiac angiosarcomas tend to affect adults at a younger age than most other cancers. They usually spread quickly and are difficult to treat because of the location of the tumor. This means people with it tend to have a poor outlook.

Early identification is key to improving your outlook with cardiac angiosarcoma. As the condition tends to run in families, regular screening for cardiac angiosarcoma may help a doctor or other healthcare professional find it early.

Keep reading to learn more about cardiac angiosarcoma, including its symptoms, diagnosis, and treatment.

The symptoms of cardiac angiosarcoma depend upon where the cancerous cells are growing.

Most cardiac angiosarcomas grow in the right atrium of your heart. This is the chamber of your heart that receives blood without oxygen. It then moves your blood to your right ventricle and lungs to receive oxygen.

Sometimes, the tumor can advance to your tricuspid valve between your right atrium and right ventricle.

Some symptoms include:

The symptoms of angiosarcoma can resemble those of many other conditions, including a heart attack or pulmonary embolism. Doctors will likely rule out these emergency conditions first.

Imaging may help a doctor identify cardiac angiosarcoma. Examples include:

A TTE is a noninvasive test that uses a special imaging probe held outside your chest to identify the chambers of your heart. A TTE helps doctors identify about 75% of all primary cardiac angiosarcomas.

Once a doctor identifies the tumor or potential tumor, they must test for the presence of cancerous cells. This may involve taking a sample of tumor tissue and some of your blood.

Who gets cardiac angiosarcoma?

Cardiac angiosarcoma accounts for less than 1% of all adult cancers. Experts estimate that it affects 0.007% of the adult population.

People tend to receive a diagnosis of cardiac angiosarcoma younger in life than they would for other cancers or heart conditions. In a 2019 study, the average age at the time of receiving a diagnosis of cardiac angiosarcoma was 44.4 years. It’s two to three and a half times more common in those assigned male at birth than those assigned female.

But it can affect anyone. A 2019 study reported the case of a 17-year-old female teen with cardiac angiosarcoma.

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Because cardiac angiosarcoma is so rare, doctors haven’t yet developed protocols as they have for more common cancer types, such as lung or breast cancer. More research and trials are needed to establish treatment standards.

Your treatment plan will depend upon the nature of your tumor and where it grows in your heart.

The preferred option is surgery, with chemotherapy beforehand. The goal of chemo is to reduce the size of your tumor before your surgery. In some cases, a doctor may use radiation therapy instead of chemo.

A surgeon may remove your tumor or a portion of it. They may also remove a thin border of healthy heart tissue around your tumor.

Removing your tumor can help enhance your quality of life. If the tumor is blocking blood flow, removing it will allow your blood to flow more freely and help your heart work more effectively. A 2019 review found that the median survival time of those who underwent surgery was 4 months longer than those who didn’t.

But surgery may not be possible if your tumor has grown a lot or is in a difficult location.

Studies have looked into the potential benefits of heart transplants for people with cardiac angiosarcoma. According to a 2016 study, the median survival of people with cardiac angiosarcoma was no different between those who received heart transplants and those who received palliative care.

Since most cardiac angiosarcomas have already spread by the time of diagnosis, surgery isn’t always an option. Other therapies that researchers have studied in various small trials include:

  • Anthracycline-based chemotherapy showed an overall survival (OS) of 9.9 months.
  • Combination chemotherapy with ifosfamide and docetaxel or doxorubicin had an OS of 17 months.
  • Pazopanib, a targeted therapy, had an OS of 9.9 months.
  • A combination of docetaxel and radiation had an OS of 16 months.

Cardiac angiosarcoma is rare. By the time most people receive a diagnosis, the cancer has already spread to their blood. It’s also often resistant to chemotherapy or radiation.

Due to these factors, the outlook for for people with cardiac angiosarcoma is often poor.

A 2019 review found that the average survival for people with cardiac angiosarcoma was about 7 months. About 35% of people with the condition were alive after 1 year and about 10% after 5 years.

A 2016 review found better results. Researchers observed a median OS of 25 months, ranging from 6 months to a few years. It also found that those who received multiple forms of treatment had better survival (36.5 months) than those who only received one type of treatment (14.1 months).

Factors that influenced survival in either review included:

  • Age: People younger than 45 years old had better survival rates.
  • Radiation: Those who had radiotherapy as part of their treatment plan had longer survival.
  • Tumor size: If the tumor was smaller than 5 cm (2 inches), survival rates were usually higher.
  • Tumor location: If the tumor was on the left side of their heart or in the lining of their heart, their survival was longer.
  • Metastasis: The more the cancer had spread at the time they received the diagnosis, the lower their survival rate was.

Resources for support

If a doctor has given you or a loved one a diagnosis of cardiac angiosarcoma, these resources may be of comfort to you:

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Researchers have identified the following risk factors for cardiac angiosarcoma:

  • chronic lymphedema
  • exposure to radiation
  • history of exposure to environmental carcinogens, such as cigarette smoke or airborne chemicals

There may also be a genetic component. A 2015 study suggested that an inherited mutation in the protection of telomeres protein 1 (POT1) gene may cause cardiac angiosarcoma. The POT1 gene codes for a protein that protects telomeres, the tips of your chromosomes.

Researchers don’t yet know if there’s a way to prevent cardiac angiosarcoma. Still, you can take steps to limit your exposure to carcinogens.

If you commonly work with chemicals, it’s important to protect yourself. Wear appropriate protective equipment and ensure ventilation is adequate.

Also, refrain from smoking and avoid exposure to secondhand smoke.

Cardiac angiosarcoma is a rare but serious type of cancer that affects your heart. Its progression is so rapid that it has usually already spread by the time you receive the diagnosis. There are cases where doctors don’t find it until after an autopsy.

If a doctor gives you a diagnosis of cardiac angiosarcoma, your treatment will likely include surgery and chemotherapy or radiation. But this depends on how much the cancer has spread and where it’s located in your heart. The outlook for people with cardiac angiosarcoma is usually poor, but it’s better with timely diagnosis and surgery.

Because it’s so rare, there’s very little research on cardiac angiosarcoma. As more research becomes available, new treatment options with better outcomes may emerge.