Understanding Primary Angiosarcoma of the Breast

"Unveiling the Mystery of Primary Angiosarcoma of the Breast"

Angiosarcoma of the Breast, Breast cancer comes in different forms, and one rare and aggressive variant is primary angiosarcoma. This type of cancer originates from the cells lining blood and lymph vessels and can develop in various body parts, including the breast. In this article, we delve deeper into the symptoms, risk factors, and available treatments for primary angiosarcoma of the breast.

What is the primary angiosarcoma of the breast?

Did you know that angiosarcoma is a rare form of cancer that originates in endothelial cells responsible for lining your blood and lymphatic vessels? This type of cancer can develop in any part of the body., but tends to occur most in the following conditions:

  • breast
  • skin
  • liver
  • spleen

Breast sarcomas occur in about 4.48 out of a million women annually. Among them, primary angiosarcoma of the breast is a highly uncommon type of breast cancer, accounting for less than 0.04% of all cases. Although it originates in the breast’s connecting tissues, primary angiosarcoma can quickly spread to the nearby skin and subcutaneous tissue, resulting in a high local recurrence rate and distant metastasis. It’s worth noting that secondary angiosarcoma of the breast is another rare form of breast cancer, and the two types should not be confused.

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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 feel
  • have irregular borders
  • not be tender or painful
  • grow rapidly

Other signs and symptoms are:

  • an area of thickened skin
  • a sensation of fullness
  • swelling

It is possible to experience skin discoloration or a bruised look on the breast and arm, which may be linked to secondary angiosarcoma of the breast.

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

Cancer development is characterized by the growth of abnormal cells that are no longer under control. When it comes to primary angiosarcoma of the breast, the exact cause is unclear, and there are not many known risk factors. While some studies suggest that there could be a genetic predisposition to developing this type of cancer, more research is required to confirm this. Exposure to cancer-causing substances may also play a part in angiosarcoma.

In general, primary angiosarcoma of the breast is more likely to affect young women with dense breasts who haven’t had breast cancer before. The typical age of onset is around 40 years, and there’s no known trigger for this condition. On the other hand, secondary angiosarcoma of the breast is usually caused by radiation therapy or chronic lymphedema due to breast cancer. The average age of onset is 70 years, and it typically happens about 10.5 years after radiation therapy.

How is primary angiosarcoma of the breast diagnosed?

A screening mammogram can detect a mass that you were unaware of. However, it’s important to note that primary angiosarcoma of the breast typically affects younger women who may not have initiated regular 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 ultrasound
  • breast MRI
  • PET scan

Although imaging tests can identify the size and location of a mass, they are inadequate to diagnose. Breast angiosarcomas have a pathological pattern that is not specific. It is difficult to differentiate them from benign lesions on imaging scans, particularly in younger people with dense breasts.

The most accurate method to diagnose breast angiosarcoma is through a breast biopsy. However, research suggests fine-needle biopsy can yield 40% false negative results. Therefore, your doctor may recommend a core needle biopsy.

Besides, other uncommon diagnoses, such as liposarcoma and fibrosarcoma, must be ruled out.

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How is primary angiosarcoma of the breast treated?

It is essential to begin treatment promptly for primary angiosarcoma as it can rapidly grow and spread. Typically, the initial course of treatment involves surgical removal of the tumor along with a clear margin surrounding it. The type of surgery required will depend on the mass size and may include breast-conserving surgery (lumpectomy) or mastectomy.

Unlike other forms of breast cancer, sarcomas of the breast do not typically spread through the lymphatic system. Consequently, removing regional lymph nodes may not be necessary, although your surgeon will make this determination on a case-by-case basis.

Because primary angiosarcoma is rare cancer, there are no rigid 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 exact 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.

Breast sarcomas differ from most other breast cancers as they do not originate from ductal breast cells. These cancer cells do not typically have hormone or HER2 receptors, which makes hormone and HER2-targeted therapies ineffective in treating them. Your oncology team will provide treatment recommendations based on the unique characteristics of your cancer.

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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 onset
  • unusual presentation compared to other types of breast cancer
  • its rarity

According to certain studies, there is a 50% chance of systemic recurrence. However, surgery, chemotherapy, and radiation may enhance disease-free survival and overall survival rates. In 2017, a study discovered 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 outlook depends on a variety of factors, such as:

  • whether cancer has spread beyond the breast at diagnosis
  • the size of the primary tumor
  • tumor grade (how fast it’s growing)
  • whether there were clear surgical margins
  • choice of treatments
  • age

It’s advisable to discuss your outlook with your oncology team. They can evaluate your circumstances and give you a personalized understanding of what to anticipate.

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Takeaway

Angiosarcoma of the breast is an uncommon and aggressive cancer that proliferates and can spread quickly. It often affects younger women who may not yet have started routine breast cancer screenings. The cause of this type of cancer is unknown. Treatment usually involves surgically removing the tumor, radiation therapy, and chemotherapy.

Talk with a doctor immediately if you notice a lump in your breast. While most breast changes are benign, having them checked out is a good idea, so you know.

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