Angiosarcoma of the Breast

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diagram of breast angiosarcoma, a rare form of breast cancer
Angiosarcoma of the Breast. Science Photo Library - ROGER HARRIS / Getty Images

Angiosarcoma of the breast is an uncommon type of breast cancer that begins in the blood or lymphatic vessels within the breast, and tends to be aggressive and spread rapidly. Accounting for only around 0.05 percent of breast cancers, there are two types of the disease. Primary angiosarcomas have no known risk factors, and often develop in young women. Less common, secondary angiosarcomas arise after a woman has had radiation therapy to the breast for a different breast cancer. Symptoms may include bruising, with redness and bruising of the breast, and less commonly a thickening or lump. Surgery is the treatment of choice for early stage tumors, though recurrence is common. For advanced tumors, chemotherapy and radiation therapy are often ineffective, but clinical trials looking at newer treatments have shown promise.

Breast angiosarcomas may also be referred to as mammary angiosarcomas, hemangiosarcomas of the breast, or lymphangiosarcomas of the breast (if lymph vessels are involved).

Types

To understand breast angiosarcomas, it's helpful to distinguish between carcinomas (most breast cancers) and sarcomas, as well as the differences between primary and secondary tumors.

Carcinomas vs Sarcomas

Most breast cancers that people are familiar with are carcinomas—cancers that begin in epithelial cells, such as those that line the ducts or lobules of the breast. Sarcomas, in contrast, are derived from myoepithelial cells, cells that make up connective tissues such as bones, muscles, and blood and lymphatic vessels.

The term "angio" means blood, and angiosarcomas are tumors that begin in blood or lymph vessels. Angiosarcomas may occur anywhere in the body, and over 90 percent of these tumors occur inr regions other than the breast. These tumors may be further defined as hemagiosarcomas (that start in blood vessel walls), or lymphagiosarcomas (that start in lymph vessel walls).

Due to the structure of these tissues, and unlike carcinomas, sarcomas do not have a pre-invasive stage, such as ductal carcinoma in situ.

Primary vs Secondary Angiosarcoma

Primary angiosarcomas of the breast account for around 80 percent of breast angiosarcomas, and are usually diagnosed in women between the ages of 20 and 40. Secondary angiosarcomas are often diagnosed in women over the age of 40, and usually occur five to ten years after radiation therapy for breast cancer (a different and unrelated tumor) is completed.

Symptoms

The symptoms of angiosarcoma can vary depending on whether it is primary or secondary, and whether it has metastasized to other regions of the body.

Primary angiosarcoma (diagnosed in a person who has never had breast cancer) may feel like a thickened area of the breast or an ill-defined lump. In some cases, the nearby skin may turn blue or reddish and appear to have a rash or to be bruised.

Secondary angiosarcoma often presents with an area of redness or a bluish appearance of the breast. There may also be swelling, or a mass in the breast. These lumps, unlike many breast cancers, are often painful.

In some cases, the first symptoms of angiosarcoma may occur due to the spread of the cancer in the body. Angiosarcomas are less likely to spread to lymph nodes than more common breast cancers, but tend to spread rapidly to distant regions of the body. Sites of metastasis may include the lungs, liver, bones, skin, other breast, brain, or ovaries.

These tumors have a high rate of recurrence, and may recur locally, in the breast, or in distant regions of the body.

Causes and Risk Factors

The exact cause of angiosarcoma is not known. With primary angiosarcomas, there are also no obvious risk factors for the disease as there are for the more common breast cancers.

Secondary angiocarcinomas may occur after radiation, but have also occurred in women who have developed lymphedema as a result of lymph node removal.

Diagnosis

A number of different tests may be done in the process of diagnosing a breast angiosarcoma, and the choice of initial testing often depends on the symptoms that are present. The process often begins with a careful medical history, in particular, noting any prior history of radiation therapy for breast cancer.

Imaging Studies

Radiological studies done to look for other types of breast cancer are often done, but the appearance can be somewhat different. Tests include:

  • Mammogram: Breast masses seen on mammography often have a fuzzy outline. Spiculated masses (lumps with spiky protrusions) are not usually seen.
  • Ultrasound: Angiosarcomas tend to show up well on ultrasound, and masses may have a bumpy appearance.
  • Breast MRI: These tumors

Biopsy

The type of breast biopsy chosen often depends on whether the tumor is primary or secondary. With primary angiosarcomas, a breast biopsy is often needed to define the tumor. With secondary angiosarcomas, a punch biopsy of the skin is often done. Once tissue is obtained, the disease can still be hard to diagnose, and a second opinion of the pathology report is often recommended.

Staging

After a breast angiosarcoma is diagnosed, staging is done by looking at the size of the tumor and degree of spread. Tests such as a PET scan or bone scan may be done to look for distant spread.

Treatments

The best treatments for angiosarcoma sometimes differ from those for more common breast cancers, and can include:

Surgery

Either breast conserving surgery (lumpectomy) or a mastectomy may be considered, depending on many factors. While one 2017 study, found that mastectomy was associated with worse overall survival than lumpectomy among people with primary angiosarcoma, another 2017 published in the New England Journal of Medicine study suggests that aggressive surgery (mastectomy) with wide local excision is often the treatment of choice with secondary angiosarcoma.

Unlike epithelial breast cancers, a sentinel node biopsy or axillary lymph node dissection is not usually done, as these tumors are much less likely to spread to regional lymph nodes.

Also unlike most breast cancers, adjuvant therapy (treatment following surgery to reduce the risk of recurrence), does not appear to be effective, including treatments such as chemotherapy, radiation, and hormonal therapies.

Chemotherapy

Unlike the majority of breast cancers that arise in epithelial cells, breast angiosarcomas do not often respond to chemotherapy. That said, for some people, depending on the characteristics of the tumor, it may be helpful.

Radiation Therapy

As with chemotherapy, some people may receive radiation therapy depending on the characteristics of the tumor. On average, however, radiation does not appear to have any survival benefit for people with primary angiosarcoma, and is associated with poorer survival in people with secondary angiosarcoma.

Clinical Trials

Fortunately, studies are looking at systemic therapies (therapies that treat a cancer anywhere in the body vs locally such as with surgery) for angiosarcoma. A 2018 study notes that treatment with VEGF inhibitors appear promising. VEGF inhibitors are a type of targeted therapy that targets specific molecular abnormalities in the growth of cancer, and in general, have fewer side effects than chemotherapy. Other treatments, such as a type of immunotherapy known as interleukin-2, anti-aniogenesis therapies (such as Avastin), and hyperthermia (using heat to kill cancer) are being studied in clinical trials.

While radiation therapy is not usually effective, the use of hyperfractionated radiation therapy is being evaluated to see if it may be of benefit in reducing recurrence.

Finding a Doctor/Second Opinion

For people who have been diagnosed with angiosarcomas, seeking treatment or at least a second opinion at a major cancer center might improve income.

A 2018 study in the British Journal of Surgery found that women with secondary angiosarcomas of the breast who were referred for specialty treatment (with a multidisciplinary team that manages sarcomas) had a lower recurrence rate and improved disease-specific survival.

The larger cancer centers, especially National Cancer Institute-designated centers, are more likely to have oncologists who specialize in sarcomas, and have experience in treating people with this uncommon form of breast cancer.

Prognosis

While angiosarcomas are usually aggressive tumors, everyone is different, and your prognosis will depend on the stage at which it is diagnosed, how far it has spread at the time of diagnosis, and how aggressively it is treated. Survival rates vary significantly among studies, but are better when the tumor can be surgically removed.

A Word From Verywell

Coping with an uncommon type of breast cancer can be challenging, as less is known and there are fewer survivors around to talk with. Fortunately, online support communities now allow people with uncommon and rare cancers the chance to communicate with others facing a similar journey around the globe. In addition, in the case of secondary angiosarcoma, people are called upon to face cancer a second time. As with any cancer, it's important to learn about your cancer and be your own advocate, but this is of utmost importance with uncommon tumors. With fewer specialists available who have experience in treating the disease, you may need to travel to a high volume cancer center to get the best care with the latest treatments and clinical trials.

Sources:

Arona, T., Terracina, K., Soong, J., Idowu, M., and K. Takabe. Primary and Secondary Angiosarcoma of the Breast. Gland Surgery. 2014. 3(1):28-34.

Lyou, Y., Barber, E., Mehta, R. et al. Radiation-Associated Angiosarcoma of the Breast: A Case Report and Literature Review. Case Reports in Oncology. 2018. 11(1):216-220.

Plichta, J., and K. Hughes. Radiation-Induced Angiosarcoma After Breast-Cancer Treatment. The New England Journal of Medicine. 2017. 376(4):367.

Srinivasan, F., singh, J., Stevenson, P. et al. Impact of Specialist Management on Survival From Radiation-Induced Sarcoma of the Breast. British Journal of Surgery. 2018. 105(4):401-409.

Yin, M., Wang, W., Drabick, J., and H. Harold. Prognosis and Treatment of Non-Metastatic Primary and Secondary Breast Angiosarcoma: A Comparative Study. BMC Cancer. 2017. 17(1):295.

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