Breast Angiosarcoma and Other Breast Sarcomas

Angiosarcoma — a cancer that begins in the cells lining the blood and lymph vessels — is rare. For every 1 million people in the United States, one will be diagnosed with an angiosarcoma each year. 

If you’ve received radiation therapy for breast cancer, your risk of developing breast angiosarcoma may be higher: A JAMA Oncology report from 2019 estimated that 1 in 1,000 people who had radiation for breast cancer went on to develop angiosarcoma in the breast or chest wall.

While the risk of angiosarcoma is low, it’s important for people who are receiving radiation for cancer to be aware of it, says Michael Wagner, MD, a medical oncologist at Dana-Farber Cancer Institute. This way they know to reach out to their doctor immediately if they notice symptoms of the disease, which include a rash or bruise on the breast that doesn’t go away.

Breast angiosarcoma is a very rare type of cancer that starts in the blood or lymph vessels in the breast. Because so few people are diagnosed with this disease, researchers are still trying to determine how best to stop it from spreading or coming back. 

There are two main types of breast angiosarcoma:

Primary breast angiosarcoma occurs in people who’ve never had breast cancer before. The causes and risk factors of primary breast angiosarcoma aren’t as well understood as angiosarcoma in people who’ve been treated for breast cancer, says Chirag Shah, MD, a radiation oncologist at Cleveland Clinic. This is mainly because of how rare this cancer is. 

Secondary (also called treatment-related or radiation-induced) breast angiosarcoma occurs in people who’ve been previously treated for breast cancer. In addition to radiation therapy, some research suggests that removal of lymph nodes and chronic lymphedema (swelling) in the breast or arm may increase the risk of breast angiosarcoma.  

Primary breast angiosarcoma most commonly develops when people are in their 30s and 40s, while secondary breast angiosarcoma tends to develop later in life (7 to 10 years after radiation), according to a 2019 review article in Translational Cancer Research.

Symptoms of primary breast angiosarcoma can include: 

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  a thickened area of skin on the breast

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  a lump in the breast

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  discolored rash or bruised appearance on the skin of the breast or arm

Symptoms of secondary breast angiosarcoma can include:

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  swelling in the breast or arm

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  a lump in the breast

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  discolored rash or bruised appearance on the skin of the breast or arm

Olutayo Sogunro, DO, a breast surgical oncologist at Johns Hopkins University, says that while many people with primary angiosarcoma often present with a mass in the breast, secondary angiosarcoma can often appear as a skin tag, pimple, or purple or blue-hued lesion on the breast. 

“A lot of times, patients end up going to the dermatologist, because they think [secondary angiosarcoma is] a skin thing,” Sogunro says, “so there's oftentimes a delay in diagnosis, because people aren't necessarily looking for it.”

If you identify a breast lump, pimple, or a bruise on your breast that’s not going away, you should contact your doctor right away. They’ll likely perform a series of tests, including a physical exam, imaging, and a biopsy to determine what is causing your symptoms.

Both primary and secondary breast angiosarcoma can be diagnosed with a biopsy of the skin or breast tissues, according to Shah. The type of biopsy will depend on your symptoms and how much skin is affected. Imaging may also be used to determine if the cancer has spread. 

Marilin Rosa, MD, a breast pathologist at Moffitt Cancer Center, says the type of imaging used will depend on the location of the lesion as well as the degree of concern the surgeon or other radiologists have. Shah says he often uses MRI or mammograms for breast imaging, and PET or CT scans to determine if the cancer has spread after the diagnosis is made. 

Because angiosarcoma is so rare, few clinical trials have been conducted, says Donald Lannin, MD, director emeritus of The Breast Center at Yale University. This has led to some debate about how best to treat the disease, he adds.

For both primary and secondary breast angiosarcomas, Shah says he will first consider chemotherapy, then surgery, then radiation. But he says there are some people who have very small angiosarcomas, who he might consider for surgery first, and then chemotherapy.

For secondary breast angiosarcomas, Sogunro first considers surgery to remove all the areas with the angiosarcoma. Most physicians agree that surgery should be the mainstay of treatment for secondary angiosarcoma, and the role of additional treatment such as chemotherapy is less well understood. 

Wagner, Shah, Rosa, and Sogunro all recommend that people with breast angiosarcomas go to specialized cancer centers familiar with treating this rare disease. In a specialized cancer center, Rosa says, “you have a team of physicians that is dedicated to that specific disease or that specific area.” This not only includes a breast surgeon that might be more skilled in excising the entire lesion, but also a radiologist, pathologist, and radiation oncologist specialized in breast cancers. 

Some cancer centers familiar with treating breast angiosarcoma include Mayo Clinic Breast Clinic, MD Anderson, and the University of Florida, according to Shah. 

Every breast cancer is different. There are lots of variables that impact a person’s chances of survival after being diagnosed with breast angiosarcoma, including factors like a cancer’s stage, the cancer grade, and a person’s lifestyle. Some research suggests that people with secondary angiosarcomas may have lower survival rates than people with primary angiosarcomas, but this may be due to the fact that people are older when they are diagnosed and the tumors tend to be more aggressive. 

Breast angiosarcoma is the most common type of breast sarcoma — or cancer that begins in the connective tissues of the breast, such as the fat, muscle, or blood vessels. 

Other types of breast sarcomas include

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  leiomyosarcomas (origin: smooth muscle)

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  liposarcoma (origin: fatty tissue)

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  fibrosarcoma (origin: tissues that wrap around tendons, ligaments or muscles)

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  rhabomyosarcoma (origin: skeletal muscles)

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  osteosarcoma (origin: bones)

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  sarcomas of uncertain differentiation (origin: unknown) 

These other breast sarcomas are even less common than breast angiosarcomas and have even more limited clinical and survival information according to a 2021 review in Sarcoma.