What are the other Names for this Condition? (Also known as/Synonyms)

• Breast Angiosarcoma
• Hemangiosarcoma of the Breast
• Secondary Angiosarcoma of the Breast

What is Angiosarcoma of Breast? (Definition/Background Information)

• Angiosarcoma of Breast is an infrequent, aggressive, and often rapidly developing tumor, without signs and symptoms in the early stages. The tumor arises from the vascular linings, which involve the blood vessels
• They are regarded as a type of high-grade malignant cancer that spreads and infiltrates the surrounding tissues and organs. “High-grade” means that the tumor is very aggressive
• The outcome for an individual with this tumor is poor, due to its high metastasizing rate. Metastasis is when the tumor leaves the site of origin and grows in another site
• Breast Angiosarcomas are seen to peak during a woman’s young-to-middle aged period. These tumors are rare and only 10% of all angiosarcomas are Angiosarcomas of the Breast
• A combination of chemotherapy (drugs), radiation therapy, and surgical procedures, are used to treat these breast tumors

There are two types of Angiosarcoma of Breast:

• Primary Angiosarcoma of the Breast - diagnosed, only when there is no previous of breast cancer
• Secondary Angiosarcoma of the Breast - these occur after a radiation therapy for breast cancer

Who gets Angiosarcoma of Breast? (Age and Sex Distribution)

• Angiosarcoma of Breast may affect a wide range of age groups, with a general peak during ages 20-40 years. Commonly, the tumor has been diagnosed in young women
• Secondary Angiosarcoma of the Breast usually occurs in older women, over 45 years (mean age 60 years)
• However, only 1 in 2,500 cases of breast cancer are Breast Angiosarcomas
• There is no known ethnic or racial preference

What are the Risk Factors for Angiosarcoma of Breast? (Predisposing Factors)

The risk of Angiosarcoma of Breast is linked to the following factors:

• A previously performed radiotherapy for breast cancer is a risk factor for Secondary Angiosarcoma of the Breast. The tumor may be radiation-induced. In such cases, it is called Radiation-Induced Angiosarcoma. The time interval for angiosarcoma development, after the radiation treatment for breast cancer, may vary from 5-10 years
• Chronic obstruction of the lymph vessels, called lymphedema, due to mastectomy in women, can increase the risk of Breast Angiosarcoma. Lymphedema occurs because of surgical removal of lymph nodes from the axillae (from under the armpits)
• The risk is greater in women, who have undergone breast cancer surgery to remove large masses (tumors) from the breast
• Chemical and environmental contaminants, like arsenic, thorium dioxide, vinyl chloride,  dioxin, and many other agents
• Presence of foreign materials in the body, like implants, graft material, polyester fiber (Dacron, from suture), etc., which are sometimes known to act as carcinogenic (cancer-causing) agents
• Angiosarcoma of Breast has also been associated to occur in patients diagnosed with neurofibromatosis (type 1), Klippel-Trenaunay syndrome, and Maffucci syndrome
• Individuals affected by immune deficiency disorders, like AIDS, have a higher risk of developing the tumor

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Angiosarcoma of Breast? (Etiology)

• The exact cause and mechanism of formation of Angiosarcoma of Breast, is unknown. They are thought to occur as a reactive process, owing to some preexisting factor(s)
• These preexisting factors may include:
  • Radiation exposure
  • Chronic lymphedema
  • Exposure to chemicals and environmental pollutants
  • Presence of foreign materials in the body for long periods (such as an implant or graft)

What are the Signs and Symptoms of Angiosarcoma of Breast?

The signs and symptoms of Angiosarcoma of Breast include:

• The breast tumor may appear as a rash, breast mass, or a thickening of the breast skin. Reddish discoloration of the skin may be observed. In some rare cases, the tumor may be painful
• The tumor may initially grow at a moderate rate, and then suddenly start progressing rapidly, during the later stages. In most cases, the initial tumor growth periods are asymptomatic
• Due to large size of tumor growth, the adjoining organs, nerves, and muscles, may be compressed or restricted. Often, these signs may be the first indications of the tumor
• In some individuals bleeding disorders, anemia (decreased red blood cells), internal bleeding, liver damage, etc. may be observed - these developments may be sudden or spontaneous
• Enlarged lymph nodes

How is Angiosarcoma of Breast Diagnosed?

The diagnostic tests vary, based on location of the tumor. A diagnosis of Angiosarcoma of Breast may involve:

• Physical examination and evaluation of patient’s medical history
• Mammogram of breast
• Breast ultrasound
• Histopathological studies conducted on a breast biopsy specimen - the specimen is examined under a microscope by a pathologist to arrive at a definitive diagnosis
• MRI scan, CT scan, to aid in obtaining a clear image of the tumor prior to surgery
• PET scans may be used to determine the extent of tumor spread in the body

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Angiosarcoma of Breast?

The complications of Angiosarcoma of Breast may include:

• Usually by the time Breast Angiosarcomas are detected, chances are that they would have proliferated and metastasized aggressively, damaging organs and tissues beyond repair
• This kind of angiosarcoma is also known to having a high recurrence rate, even after surgical excision and removal
• Blood loss during invasive treatment methods may be heavy
• Damage of vital nerves, blood vessels, and surrounding structures  during surgery
• Side effects from chemotherapy (toxicity), radiation therapy

How is Angiosarcoma of Breast Treated?

The treatment of Angiosarcoma of Breast is undertaken as:

• Any combination of chemotherapy, radiation therapy, and invasive procedures (surgery), are used to treat Breast Angiosarcomas
• Embolization (causing the blood in the vessels to clot) is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• Wide surgical excision of the breast tumor with removal of the entire lesion is the standard treatment mode
• When Breast Angiosarcoma is unsafe for surgical intervention; non-invasive procedures, such as chemotherapy and radiation therapy are adopted
• Post-operative care is important: Minimum activity is allowed, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Angiosarcoma of Breast be Prevented?

• There is no definitive method to prevent Breast Angiosarcoma occurrence; consider general precautions that decrease the incidence of cancer in the body
• Adopting a lifestyle that avoids prolonged exposure to pollutants and carcinogens, either at home or at work, may help in preventing Angiosarcoma of Breast
• Due to its high metastasizing potential and high recurrence rate, regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already been treated for this tumor

What is the Prognosis of Angiosarcoma of Breast? (Outcomes/Resolutions)

• Breast Angiosarcomas are rare and these high-malignancy cancers have not yet been completely understood. The long-term prognosis depends on a combination of factors, such as:
  • Age of the individual
  • Tumor stage at detection
  • Tumor size and location
  • Its ki-67 value - a protein found in cells that is a good indicator of how fast the tumor cells are growing. The ki-67 value is determined by a pathologist and is usually mentioned in the pathology report
  • Patient’s response to treatment and medical therapy
• Given the high rate of recurrence and metastasis, the long-term outcome (5-year) is poor for most individuals. Generally, secondary tumors do much worse than primary tumors
• The best treatment practice for Angiosarcoma of Breast involves a combination of radiotherapy with surgical intervention

Additional and Relevant Useful Information for Angiosarcoma of Breast:

Angiosarcomas can occur anywhere in the body; the most common tumors include the following:

• Angiosarcoma of skin
• Angiosarcoma of breast
• Angiosarcoma of spleen
• Angiosarcoma of liver
• Angiosarcoma of soft tissue