The use of binary terms such as “male” and “female” or “men” and “women” in this article reflects the language of the sources we’ve used. Unless otherwise noted, it’s unclear whether the research we reference included participants with expansive gender identities.

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 is a rare clinical condition, making up for approximately 0.04% of all malignant breast cancer cases. It tends to be more common in people assigned female at birth, but all people may develop 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 endothelial cells or cells that line blood vessels and lymph vessels.

Angiosarcoma of the breast is characterized by rapid growth and spread. Owing to its rarity, it has an unfavorable outlook due to the lack of established treatment guidelines.

There are two types of angiosarcoma of the breast:

Primary angiosarcoma

BA is an aggressive and poorly prognostic tumor, usually diagnosed in women aged 30–50 without a history of cancer or identifiable risk factor

Primary breast angiosarcoma is an aggressive form of cancer, commonly occurring in women aged 30 to 50 without a history of cancer or known risk factors.

t usually presents with an indefinite lump or mass, begins in the breast tissue, and then enters nearby skin and subcutaneous tissue.

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 people in their 60s and 70s. It often arises around 7 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:

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

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.

Surgery

Doctors typically recommend removing part of the breast or the entire breast. They typically do not remove axillary lymph nodes. These are a cluster of lymph nodes in the armpits.

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 following surgery. Radiation treatment secondary to surgery may enhance outcomes but further research into its efficacy is necessary.

The use of radiation therapy to treat radiation-induced angiosarcoma is controversial, although extremely limited cases show promise.

Chemotherapy

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.

Receiving a diagnosis of breast angiosarcoma can be difficult. As a result, the outlook for both primary and secondary angiosarcoma of the breast is often unfavorable.

Survival rate

Different studies report that the relative 5-year survival rate is between 28% and 54%. Relative 5-year survival rate estimates the percent of patients that will be alive at 5 years post-diagnosis compared to people without the disease.

Primary vs. secondary

Typically, the outlook for secondary breast angiosarcoma is less favorable than that for primary, but further research needs to be performed to confirm this.

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.