Angiosarcoma is a rare type of soft tissue cancer that develops in the lining of blood and lymph vessels. While it can occur anywhere in the body, angiosarcoma most often occurs in the skin, breast, liver, and spleen.

Angiosarcomas grow very fast. They require prompt and aggressive treatment.

This article takes a closer look at angiosarcoma, including symptoms to look for, causes, treatment, and outlook.

Your blood and lymph vessels (which are part of the immune system) are lined with a single layer of cells called the endothelium. Angiosarcoma begins when endothelial cells grow and multiply abnormally to form a tumor.

Although angiosarcoma can occur in any part of your body, it most commonly affects:

  • the skin of the head or neck
  • the skin of the breast or torso
  • liver
  • heart

Angiosarcoma vs. sarcoma

Angiosarcoma is a type of sarcoma that affects your soft tissue. Your soft tissue includes:

There are more than 70 types of sarcoma. Types of soft tissue sarcomas include:

Angiosarcoma is very rare. Only 1% to 2% of all soft tissue sarcomas, which are already rare, are angiosarcomas.

In the United States, for every 1 million people, 1 person is diagnosed with angiosarcoma each year. It can happen at any age but is more common in people over 70.

Out of all the places in your body where angiosarcoma can develop, the skin of the head and neck is the most common location.

Angiosarcoma begins due to a mutation (an error in your DNA) in one of the endothelial cells in your blood or lymph vessels. This causes the unhealthy cell to grow and multiply quickly, eventually creating a tumor.

Researchers don’t know what exactly causes these mutations. But they do know certain risk factors can increase the risk of angiosarcoma, such as:

Symptoms of angiosarcoma depend on where the tumor is located.

Angiosarcoma that affects your skin may have the following symptoms:

  • a bruise or lesion that doesn’t heal
  • a raised area of skin that grows larger over time
  • a lesion that bleeds when scratched or bumped
  • a soft lump surrounding a lesion

Angiosarcoma that affects other organs, like the heart or liver, may not cause any symptoms. When it grows large enough, it can start causing pain in these organs.

Your doctor will perform a thorough physical exam to understand your condition. They may ask questions about your medical history and conditions that run in your family. In addition, they will likely run the following tests:

  • imaging studies, such as X-ray, CT, MRI, or PET scans
  • a biopsy, which involves taking a small sample from the tumor with a needle

A biopsy is the only test that can confirm a diagnosis of angiosarcoma. During the biopsy, a specially trained doctor studies the tumor sample in a microscope to determine the type and stage of cancer.

Angiosarcoma is a fast-growing cancer, so your healthcare team will treat it aggressively. Treatment depends on whether it has spread to other organs or tissues in your body.

Angiosarcoma that hasn’t spread is most often treated with surgery. Chemo or radiotherapy can sometimes be administered before or after the surgery.

If angiosarcoma has spread to other parts of your body, the main treatment approach will likely be chemotherapy.

Other approaches include:

The best way to prevent angiosarcoma is to avoid risk factors associated with this cancer.

Not all risk factors can be avoided. Examples include genetic predisposition and radiotherapy to treat other conditions.

However, you may reduce your risk by protecting your skin from the sun and limiting your exposure to certain chemicals, like vinyl chloride, arsenic, or thorium dioxide.

Angiosarcoma is a very aggressive, fast-growing tumor. Because it starts in your blood or lymph vessels, it can spread very quickly to other tissues and organs. Once it’s spread, angiosarcoma is very difficult to treat.

According to the American Cancer Society, the relative 5-year survival rate for soft tissue sarcomas that have not spread is 85%.

If the tumor spreads, the survival rate is 65% for cancer that has spread into nearby lymph nodes. The survival rate is 15% for cancer that has spread into distant organs.

However, your situation — in terms of how far the cancer has spread and how your body responds to treatment — is unique. Be sure to talk with your doctor to find out about your individual outlook.

Angiosarcoma is a rare but aggressive type of cancer. It affects your blood and lymph vessels. It’s most often found on the skin of the head and neck.

Angiosarcomas of the skin may look like a lesion or bruise that doesn’t heal with time.

There are several risk factors for this condition, including genetics and exposure to radiation and certain chemicals.

This cancer can spread quickly into other organs and tissues, which reduces survival rates. But newer treatments for angiosarcoma, such as targeted medications and immunotherapy, are promising and may extend people’s outlook.