Angiosarcoma is a rare type of cancer that forms in the inner walls of your blood vessels or lymphatic vessels. It can grow in any part of your body, including the soft tissues of the breast.

Angiosarcoma cancer can be aggressive and may spread to other parts of your body. It's difficult to diagnose and manage due to how rare it is.

If you have an angiosarcoma diagnosis, our breast cancer specialists at Aurora Breast Centers are here for you every step of the way. We provide the latest diagnostic tests, including genetic testing, and treatments for angiosarcoma. You can trust us to provide the most comprehensive cancer care available in Wisconsin.

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What is angiosarcoma breast cancer?

Angiosarcoma is a very rare type of cancer that represents only about 0.1% to 0.2% of all cases of breast cancer. It can affect both women and men. Angiosarcoma can occur spontaneously or may develop as a complication of previous radiation therapy to the breast.

Since angiosarcoma forms in your blood or lymphatic vessels, it can develop in any part of your body. Besides the breast, it can form in your head and neck, chest and in your liver or spleen.

Types of angiosarcoma breast cancer

There are two main types of angiosarcoma of the breast:

  • Primary angiosarcoma: This is a type of angiosarcoma that develops without any known factors or previous radiation therapy. You may have symptoms such as a rapidly growing mass in your breast and. Primary angiosarcoma is more common in younger women, typically between 30-50 years old.
  • Secondary angiosarcoma: This type of angiosarcoma develops because of an existing condition. More commonly, it develops after radiation therapy treatment. It can also develop due to exposure to certain chemicals or a complication from getting breast implants. Cancer cells usually develop 8-10 years after radiation treatment.

An early sign of either type of angiosarcoma is a lump or mass in the breast that grows rapidly – a common sign with other types of breast cancer. However, angiosarcoma is often diagnosed at a more advanced stage due to its rarity and similar symptoms with other breast cancers. This stresses the importance of early detection and screenings.

How aggressive is angiosarcoma breast cancer?

Angiosarcoma can be aggressive. Your prognosis depends on several factors, including the stage of the cancer, the size and location of the tumor, whether it's primary or secondary and the response to treatment.

Angiosarcoma tends to have a poorer prognosis compared to more common types of breast cancer, such as invasive ductal carcinoma (IDC).

Cancer tumors can grow quickly and may invade surrounding tissues, making them more difficult to treat. Angiosarcoma also has a high risk of recurrence, even after aggressive treatment with surgery, radiation therapy and chemotherapy.

Risk factors for angiosarcoma breast cancer

While the exact cause of angiosarcoma breast cancer is unknown, there are several risk factors that increase your likeliness of developing it.

Risk factors include:

  • Radiation therapy: Exposure to radiation, especially high doses or repeated treatments, is a significant risk factor for developing angiosarcoma.
  • Environmental exposures: Exposure to certain chemicals, such as vinyl chloride or arsenic, has been associated with an increased risk of angiosarcoma.
  • Chronic inflammatory conditions: Certain chronic inflammatory conditions, such as lymphedema or chronic lymphatic stasis, may increase your risk of developing angiosarcoma.
  • Genetic factors: While rare, a family history of breast cancer may increase your risk of angiosarcoma.

These factors may increase the risk of developing angiosarcoma. However, if you have any risk factors you may never develop angiosarcoma. It may also occur if you have no known risk factors.

Symptoms of angiosarcoma breast cancer

Angiosarcoma symptoms vary depending on the type and location of the tumor. The most common symptom is a noticeable lump or mass under the skin or in deeper tissues that may be painful.

Other symptoms of angiosarcoma breast cancer may include:

  • Skin changes such as reddish or bluish discoloration
  • Swelling in the affected area
  • Pain
  • Bleeding
  • Fatigue

If you notice any unusual lumps, skin changes or other symptoms that get worse over time, schedule an appointment with your doctor.

Grades of angiosarcoma breast cancer

Your angiosarcoma grade is determined by the characteristics of cancer cells. When examined under a microscope, your care team can better understand your cancer and potentially predict the speed at which your cancer cells may grow and spread to nearby tissue or other parts of your body.

Angiosarcoma breast cancer is graded on a scale ranging from one to three:

  • Grade 1 (low grade): Cancer cells closely resemble normal breast tissue cells. They exhibit slow growth and spread, rendering this grade less aggressive.
  • Grade 2 (intermediate grade): Cancer cells show some abnormal characteristics but are not visibly different from normal cells. They may grow and invade other areas of the body at a moderate pace.
  • Grade 3 (high grade): Cancer cells appear significantly different from normal cells. They demonstrate aggressive behavior, including rapid growth and spread, often leading to a poorer prognosis.

Due to angiosarcoma being difficult to diagnose or catch in early stages, it’s often already at a grade3 by the time it’s detected.

Stages of angiosarcoma breast cancer

The stage of angiosarcoma depends on the grade and location of the tumor. The four stages of angiosarcoma cancer include:

  • Stage 1: The tumor is small and localized, with no evidence of spreading to nearby lymph nodes or distant organs.
  • Stage 2: The tumor is larger or has spread to nearby tissues, but there is still no evidence of spread to lymph nodes or distant organs.
  • Stage 3: The tumor may be any size and may or may not have spread to nearby lymph nodes, but there is evidence of distant spreading to organs such as the lungs, liver, bones or other places.
  • Stage 4: The cancer has spread extensively to distant organs or tissues, making it difficult to treat and usually indicating a poorer prognosis.

Diagnosing angiosarcoma breast cancer

Angiosarcoma is typically diagnosed through a physical examination, imaging tests and biopsy. Mammograms are not usually effective in detecting angiosarcoma.

Your provider will check your symptoms, medical history and discuss any risk factors you may have for angiosarcoma. During the physical exam, they may feel any lumps or masses.

Tests used to diagnose angiosarcoma may include:

  • Imaging tests: Imaging studies such as ultrasound, MRI, CT scan or PET scan may be performed to visualize the tumor and assess its size, location and extent of the spread.
  • Biopsy: If a suspicious mass or lesion is identified, a biopsy will be performed to obtain a tissue sample for examination under a microscope. This is the most definitive way to diagnose angiosarcoma. Depending on the location of the tumor, the biopsy may be performed using a needle (needle biopsy) or by surgically removing a sample of tissue (incisional or excisional biopsy).
  • Histological examination: A pathologist will examine the tissue sample obtained from the biopsy to look for characteristic features of angiosarcoma, such as abnormal blood vessel formation and atypical cells.

Depending on the location and extent of your tumor, additional tests such as blood tests, lymph node biopsy or imaging studies of other parts of the body may be needed to determine if the cancer has spread.

Once a diagnosis of angiosarcoma is confirmed, other tests will determine the stage of cancer and guide treatment decisions.

Angiosarcoma breast cancer treatments

Treatment for angiosarcoma depends on various factors, including the tumor's location, its size, grade, stage and your overall health.

Some common treatment options for angiosarcoma include:

  • Surgery: Sugery is the primary treatment for angiosarcoma. Options for angiosarcoma include a mastectomy, which involves the partial or full removal of the breast tissue, or a lumpectomy (breast conserving surgery). Your surgeon may also perform a lymph node dissection to remove nearby lymph nodes.
  • Radiation therapy: If you have a lumpectomy, your doctor will give you radiation therapy to kill any remaining cancer cells in your breast and reduce the risk of it returning. In some cases, a mastectomy might be followed by breast reconstruction surgery.
  • Chemotherapy: Chemotherapy may be used for angiosarcomas that are more aggressive and have spread to other parts of the body.
  • Targeted therapy: Some angiosarcomas may include specific molecules that can be targeted by certain medications that impact the formation of new blood vessels to slow down the growth of the tumor growth.

Your care team at Aurora Health will use a collaborative approach to provide the best treatment possible. Supportive care services such as counseling, nutrition therapy, palliative care and physical therapy may also be offered to help you manage the physical and emotional challenges of cancer treatment.

Is angiosarcoma breast cancer curable?

Angiosarcoma of the breast can be challenging to cure due to its aggressive nature and tendency for recurrence. Even with aggressive treatment, the risk of local recurrence or distant spreading remains relatively high.

For cases where the angiosarcoma has spread to other organs, treatment may focus on controlling the disease and managing your symptoms. Chemotherapy, targeted therapy and other systemic treatments may be used to help slow the progression of the disease and improve your quality of life.

Are you at risk for breast cancer?

Knowing your chance of developing breast cancer can help you plan a routine screening schedule. Our breast health quiz estimates your five-year and lifetime risk and gives you an idea of what to do next based on your results.

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