Angiosarcoma of the breast is a rare, aggressive form of breast cancer. It grows rapidly and often has an unfavorable outlook due to its rarity.

Angiosarcoma is a rare type of cancer that starts in the cells that line the blood vessels and lymph vessels. While it can occur anywhere in the body, it often affects the breasts, spleen, and liver vessels.

Angiosarcoma of the breast makes up less than 0.05% of people with malignant breast cancer. It tends to be more common in women, but men may also get it.

In this article, we discuss angiosarcoma of the breast, its symptoms, and outlook.

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Angiosarcoma of the breast is an uncommon form of breast cancer. Unlike the most common types of breast cancers that begin in ducts or lobules, this disease begins in epithelial cells or cells that line blood vessels and lymph vessels.

The rapid growth and spread characterize angiosarcoma of the breast. It has an unfavorable outlook due to the lack of established treatment guidelines, owing to its rarity.

There are two types of angiosarcoma of the breast:

Primary angiosarcoma

This type occurs in people who never received treatment for breast cancer. It usually presents with an indefinite lump or mass, begins in the breast tissue, and then enters nearby skin and subcutaneous tissue. It tends to commonly affect younger women in their 30s and 40s.

Secondary angiosarcoma

Secondary angiosarcoma develops due to receiving radiation therapy to treat breast cancer. It is also associated with long-standing lymphedema in the arm and breast, known as Stewart-Treves syndrome.

It is more common than primary angiosarcoma and tends to affect older women between the average ages of 6771. It often arises about 5–10 years after radiotherapy treatments.

Symptoms of angiosarcoma of the breast depend on the type and whether it spreads to other body areas.

Symptoms may appear subtle and people may confuse the disease with nonmalignant skin conditions such as rosacea, angiomas, or bruising.

Primary angiosarcoma symptoms

The symptoms of primary angiosarcoma include:

Secondary angiosarcoma symptoms

The symptoms of secondary angiosarcoma include:

The outlook for both primary and secondary angiosarcoma of the breast is often unfavorable. According to a 2017 study involving 472 participants living with angiosarcoma of the breast, the 5-year survival rate for primary angiosarcoma is 44.5%. For secondary angiosarcoma, the 5-year survival rate is much lower, at 22.5%.

The study found that age and tumor spread were strong predictors of low survival rates in secondary and primary angiosarcoma.

The timing of finding the cancer is also a factor, in that the earlier the tumor discovery, then the better the outlook.

The above study showed a more locally advanced tumor stage and high spread of tumors in participants with secondary angiosarcoma. These results suggest the secondary type of this cancer has more aggressive characteristics and spreads faster.

However, a small 2020 study found that participants with secondary angiosarcoma had better responses and survival rates than those with primary.

Recurrence is also common despite tumor removal. However, complete removal of the tumor showed better results than incomplete removal.

A doctor may order several tests to look for breast changes, including location, size, and extent. Imaging tests include:

A small 2014 study found that the imaging results from mammograms and ultrasounds for angiosarcoma of the breast are nonspecific compared with MRI results.

A breast biopsy is the primary diagnostic method for breast cancer, where a doctor removes a piece of tissue. It can come from a tumor deep in the breast.

Healthcare professionals may recommend a fine needle aspiration or core needle biopsy for primary angiosarcoma. However, they may also advise a skin punch or incisional biopsy if the affected area is on the outer skin surface of the breast, typical in secondary angiosarcoma.

In a lab, medical professionals analyze the sample by viewing it under a microscope for diagnosis based on changes in cell structure. They can stain samples with markers such as CD31 and CD34, highly sensitive markers for vascular tumors, which are cancerous tumors formed from blood vessels or lymph vessels.

Angiosarcomas grow and spread rapidly, requiring urgent and aggressive treatment. But because the disease is so rare, there is currently no consensus on the best treatment overall.


Doctors typically recommend removing part of the breast or the entire breast. They typically do not remove axillary lymph nodes.

However, a 2017 study found that mastectomy was more associated with unfavorable outcomes than breast conservation surgery in people with primary angiosarcoma.

Radiation therapy

People with primary angiosarcoma may receive radiation therapy, but it is not as effective for those with secondary angiosarcoma.

The same 2017 study found that people with secondary angiosarcoma had lower overall survival when receiving adjuvant radiation.


Doctors may also give chemotherapy or targeted drugs, but the medications may differ from those used in common types of breast cancer.

A 2019 research review described genetic mutations and altered protein expressions in the disease, showing that targeted therapy can potentially be more effective than traditional chemotherapy.

Clinical trials

There are ongoing clinical trials to look for more effective treatments for angiosarcoma of the breast. Current clinical trials involve combinations of cytotoxic chemotherapies, immunotherapies, and targeted therapies.

Angiosarcoma of the breast is a rare and aggressive type of breast cancer. It may appear on its own, or it may occur from getting radiation therapy to treat breast cancer.

Its symptoms are nonspecific, making it difficult for people to distinguish it from nonmalignant skin conditions. Getting a biopsy is typically the best way to diagnose the condition.

Due to its rarity, there are currently no established guidelines for its treatment. However, treatment options such as mastectomy, radiation, and chemotherapy, are available. Clinical trials are ongoing for immunotherapies, targeted therapies, and cytotoxic chemotherapies.