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Understanding Primary Angiosarcoma of the Breast

Medically reviewed by Faith Selchick, DNP, AOCNP, Nursing, Oncology — By Ann Pietrangelo on October 17, 2022Primary angiosarcoma of the breast is a rare and aggressive kind of breast cancer. Angiosarcoma is a type of cancer that starts in cells that line blood and lymph vessels. It can occur anywhere in the body, including the breast. This article takes a closer look at primary angiosarcoma of the breast, including symptoms, risk factors, and treatment.

What is primary angiosarcoma of the breast

Angiosarcoma is a rare type of cancer that develops in endothelial cells. Those are the cells that line your blood and lymphatic vessels. This type of soft tissue cancer can develop in any part of the body, but tends to occur most in the:breastskinliverspleen The annual incidence of all breast sarcomas is about 4.48 cases per million women. Primary angiosarcoma of the breast is extremely rare, representing less than 0.04% of all breast cancers. While it starts in the connective breast tissues, primary angiosarcoma can quickly grow and spread into nearby skin and subcutaneous tissue. It has a high rate of local recurrence and distant metastasis. Another incredibly rare breast cancer is called secondary angiosarcoma of the breast. The names are similar, but these are two distinct conditions.

What are the symptoms of primary angiosarcoma of the breast

Like most breast cancers, one of the main signs is a breast lump. The lump is likely to:be easy to feelhave irregular bordersnot be tender or painful grow rapidly Other signs and symptoms are:an area of thickened skina sensation of fullnessswelling There can also be some skin discoloration or a bruised appearance of the breast and arm. But this is more likely to be associated with secondary angiosarcoma of the breast.

What are the causes and risk factors for angiosarcoma of the breast

Cancer begins with out-of-control growth of abnormal cells. What kicks off this process in primary angiosarcoma of the breast is not clear and risk factors are largely unknown. Angiosarcoma can sometimes run in families. Some research suggests a possible genetic predisposition to develop this type of cancer, but more studies are needed in this area. Exposure to cancer-causing chemicals may also play a role in angiosarcoma. Primary angiosarcoma of the breast is most likely to affect a young woman with dense breasts and no history of breast cancer. The median age at onset is 40 years. There is no known trigger. Secondary angiosarcoma of the breast is caused by previous radiation treatment or chronic lymphedema due to breast cancer. It has a median onset age of 70 years, and generally happens about 10.5 years after radiation therapy.

How is primary angiosarcoma of the breast diagnosed

A screening mammogram may pick up on a mass you didn’t know was there. But primary angiosarcoma of the breast tends to occur in younger women who may not have started routine breast cancer screening mammograms. If you have a breast lump or other signs of breast cancer, your doctor will likely perform a clinical exam, followed by a diagnostic mammogram. Other imaging tests may include:breast ultrasoundbreast MRIPET scan While imaging tests can identify the general size and location of a mass, it’s not enough to make a diagnosis. Breast angiosarcomas have a nonspecific pathological pattern. They can appear much like benign lesions on imaging scans, particularly when it involves younger people with dense breasts. The only way to accurately diagnose breast angiosarcoma is with a breast biopsy. Some research suggests that fine-needle biopsy leads to 40% of false negative results, so your doctor may recommend a core needle biopsy. Other rare diagnoses, such as liposarcoma and fibrosarcoma, must be excluded.

How is primary angiosarcoma of the breast treated

Primary angiosarcoma grows and spreads quickly, so it’s best to start treatment right away. The first treatment is typically surgery to remove the tumor plus a clear margin around it. Depending on the size of the mass, this means breast conserving surgery (lumpectomy) or mastectomy. Unlike other types of breast cancer, sarcomas of the breast don’t usually spread through the lymphatic system. So, your surgeon may not need to remove regional lymph nodes, but this is decided on a case-by-case basis. Because primary angiosarcoma is such a rare cancer, there are no hard guidelines for the next steps. Postsurgical treatment may include:Radiation therapy: Radiation is a targeted treatment to address any cancer cells that remain after surgery. This can help prevent a new tumor from forming in the same location.Chemotherapy: Chemotherapy is a systemic treatment that can destroy cancer cells anywhere in the body. This can help prevent tumors from forming in regional or distant sites. Sarcomas don’t develop from ductal breast cells, like most other breast cancers. These cancer cells are unlikely to have hormone or HER2 receptors. That means that hormone and HER2-targeted therapies used to treat other types of breast cancer may not be helpful. Your oncology team will make treatment recommendations based on the specifics of your cancer.

What s the outlook for people with primary angiosarcoma of the breast

Primary angiosarcoma generally has an unfavorable outlook. This is often due to a delay in diagnosis due to:young age at onsetunusual presentation compared to other types of breast cancerits rarity Some research suggests that the probability of systemic recurrence is 50%. But surgery followed by chemotherapy and radiation may improve disease-free survival and overall survival. A 2017 study found that the 5-year survival rate for primary angiosarcoma of the breast is 44.5%. Because the condition is so uncommon, studies and statistics are lacking. Your own outlook depends on a variety of factors such as:whether cancer has spread beyond the breast at diagnosisthe size of the primary tumortumor grade (how fast it’s growing)whether there were clear surgical marginschoice of treatmentsage It’s a good idea to discuss outlook with your own oncology team. They can assess your situation and give you a more personal idea of what to expect.

Takeaway

Primary angiosarcoma of the breast is characterized by rapid growth and a strong ability to spread. It’s a rare, aggressive cancer with no clear cause. It tends to affect younger women, possibly before they start routine screening for breast cancer. Treatment for primary angiosarcoma typically involves surgical removal of the tumor, which may be followed by radiation therapy and chemotherapy. If you notice a lump in your breast, talk with a doctor as soon as possible. While most breast changes are benign, it’s a good idea to have them checked out so you know for sure. Last medically reviewed on October 17, 2022

How we vetted this article

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ncbi.nlm.nih.gov/pmc/articles/PMC5408408/Our experts continually monitor the health and wellness space, and we update our articles when new information becomes available. Current Version Oct 17, 2022 By Ann Pietrangelo Edited By Willow Banks Medically Reviewed By Faith Selchick DNP, APRN, AOCNP Copy Edited By Megan McMorris Share this articleMedically reviewed by Faith Selchick, DNP, AOCNP, Nursing, Oncology — By Ann Pietrangelo on October 17, 2022

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