Angiosarcoma

January 22, 2026

This page was reviewed under our medical and editorial policy by Lee Cranmer, M.D., Ph.D., professor, Department of Medical Oncology & Therapeutics Research, City of Hope^® Cancer Center Duarte.

Angiosarcoma is a cancer that develops in the blood vessels or lymph vessels. It is very rare, affecting only one in a million people and accounting for around 2% of all sarcomas — the term for cancers that occur in the soft tissues of the body, such as muscle, fat, nerves, blood vessels or cartilage. People aged 70 and over are more likely to develop angiosarcoma.

What Is Angiosarcoma?

Angiosarcoma is a very aggressive cancer type that is found in the lining of the blood or lymph vessels. Although it may affect many different parts of the body, around half of all angiosarcoma tumors are found in the skin. Other common locations are the breast, the liver and the spleen.

Types

In most cases, doctors will diagnose the type of angiosarcoma someone has based on where it is found in their body. The most common types of angiosarcoma are listed below.

Skin (or cutaneous) angiosarcoma: This type develops in the layers that make up the skin, most often the head and neck, or the breast. This may be associated with exposure to the ultraviolet rays of the sun.

Liver (or hepatic) angiosarcoma: This develops in the soft tissue of the liver.

Breast angiosarcoma: This may develop in the lymph or blood vessels of the breast and is sometimes related to previous exposure to radiation therapy.

Heart (or cardiac) angiosarcoma: This develops in the heart’s blood vessels and may spread to the muscle of the heart.

Keep in mind that this disease may affect any part of the body. It is sometimes found in the spleen, bone or other soft tissues. When it is found in multiple areas, it is primarily because it has spread from one part of the body to the other, a process called metastasis.

Causes and Risk Factors

The exact causes of angiosarcoma are not fully understood in all cases. However, there are certain risk factors that may make it more likely someone will develop the disease. These include those listed below.

Ultraviolet light from the sun: Angiosarcoma may be a form of skin cancer caused by the ultraviolet rays from the sun. This type of angiosarcoma often occurs on the scalp or face.

Previous exposure to radiation therapy: People who have received radiation therapy in the past – in most cases as treatment for other types of cancer — have a higher risk of developing angiosarcoma. When this occurs, it may be even 10 to 30 years after the radiation treatment.

Having chronic lymphedema: Lymphedema occurs when the lymphatic vessels, most often in the leg or arm, are damaged. This may be due to surgery, whether for cancer or other purposes, receipt of radiation therapy (typically for cancer treatment) or other reasons. This results in the inability of lymph fluid to be removed from the extremity. Lymph fluid might be thought of as fluid that leaks out of blood vessels, because blood vessels are not normally completely sealed. The lymphatic system serves to remove this fluid and put it back in the bloodstream, much like a sump system in the basement of a house.

People with long-term lymphedema and swelling of their extremity may be more likely to develop angiosarcoma. This typically occurs only after lymphedema has been present for many years, or even decades.

Exposure to certain chemicals: Some evidence suggests that people who have been exposed to certain herbicides, arsenic or vinyl chloride (a chemical in some plastics) are at higher risk of angiosarcoma. Vinyl chloride, in particular, is associated with development of angiosarcoma in the liver.

Gene mutations: Although rare, changes to certain genes in the body may increase someone’s chances of getting angiosarcoma. When this occurs, the disease may occur within families.

Angiosarcoma Symptoms

Angiosarcoma classically causes a reddish or purplish spot or area on the skin, at least when it appears within the skin. It may swell in size and the patient may develop pain or tenderness at the site. If angiosarcoma arises internally, such changes in the skin may not occur.

Symptoms of angiosarcoma vary in different people. Symptoms may include:

• Pain, bruising or tenderness, which are common symptoms of skin angiosarcoma
• A sore abdomen, weight loss, jaundice and fatigue, which are frequent symptoms of liver angiosarcoma
• Chest pain, heart palpitations and shortness of breath, which may be signs of heart angiosarcoma

Keep in mind that angiosarcoma is a very unusual condition and the symptoms above could also be symptoms of many other, much more common conditions. Patients who experience these symptoms should discuss them with a health care provider.

Tests for Diagnosing

Cancer doctors may recommend one or several tests to accurately diagnose angiosarcoma and find out how far the cancer has spread. These often include:

A biopsy is a procedure to remove a small sample from a tumor to examine it for cancer cells. Various tests may be performed on this specimen to better characterize it. This is the primary way that angiosarcoma, or any cancer for that matter, is diagnosed.

Imaging tests, such as magnetic resonance imaging (MRI) or computed tomography (CT) scans, may help determine the location, size and extent of the cancer.

Patients may undergo other tests, depending on what type of angiosarcoma they have. If the angiosarcoma is arising in the skin, or is from an unknown site, a full skin examination may be performed by a dermatologist to identify where the disease may have arisen.

Stages

There are different forms of angiosarcoma, as noted above. Therefore, no single system of staging applies to all patients. Assignment of a stage is normally based on the location where angiosarcoma arises. One simple way of thinking about staging of angiosarcoma is how extensive the disease is. If the disease remains localized to one specific area of the body and could potentially be removed by surgery, that may be considered localized disease. If the disease is so extensive that it cannot be removed by surgery, then it may be considered advanced or disseminated disease. The latter form of the condition is normally treated with medicines, rather than surgery, as the primary treatment.

Angiosarcoma Treatment

Early treatment of angiosarcoma is important. This disease may spread rapidly in some patients. Treatment will be optimized to achieve the best possible outcome.

Surgery

If it is possible to remove an angiosarcoma tumor surgically, this is often a primary treatment option. Sometimes, it is combined with radiation therapy, chemotherapy or immunotherapy to treat the condition.

Radiation Therapy

Radiation therapy may be used after surgery to target any remaining cancer cells and to reduce the risk of angiosarcoma coming back. For tumors that are easy to remove surgically, radiation therapy may be an alternative treatment choice. In other cases, angiosarcoma may not be able to be removed surgically but is still in a local part of the body. In such cases, radiation therapy may be used as the primary treatment for the condition. Sometimes, chemotherapy is given along with radiation therapy to enhance the effect of the radiation therapy.

Chemotherapy

If angiosarcoma has spread to other areas of the body, doctors may recommend chemotherapy, which targets and kills cancer cells. Chemotherapy may also be used in combination with radiation therapy, if a tumor cannot be removed during surgery.

Immunotherapy

In some cases, immunotherapy may be used to treat angiosarcoma. This means administering medicines, most commonly intravenously, in order to recruit the immune system to attack the cancer. This is especially the case in angiosarcoma arising in the skin. This type of therapy may be used alone or combined with chemotherapy or radiation therapy to treat the condition.

Who Treats Angiosarcoma?

When receiving treatment for angiosarcoma, patients work with an expert team that includes doctors, nurses and other specialists in soft tissue sarcoma, including:

• Medical oncologists specializing in cancer drugs and medications such as chemotherapy and immunotherapy
• Surgical oncologists and orthopedic surgeons, who are experts in removing tumors that affect the soft tissues and bones of the body
• Radiation oncologists, who specialize in recommending and delivering radiation therapy
• Pathologists, to evaluate biopsy results and monitor treatment
• Supportive care providers, who may help patients manage the physical and emotional side effects of cancer and its treatments
• Dermatologists, who are experts in diseases of the skin, and may be involved when patients have cutaneous angiosarcoma
• Gastroenterologists, who specialize in treating diseases of the digestive system, and may address diagnosis and treatment for patients with hepatic angiosarcoma
• Cardiologists, who are experts in treating diseases of the heart, and may be involved when patients have cardiac angiosarcoma
• Women’s health providers, who may help address diagnosis and treatment options for patients with breast angiosarcoma

Angiosarcoma Life Expectancy

The overall five-year survival rate for angiosarcoma is 24%, which is lower than some other types of sarcoma. However, it is a very rare cancer type and each patient’s prognosis is different, depending on the location of their cancer, how far it has spread and how they respond to treatment. Experts are learning much more about the treatment of angiosarcoma, and treatment is improving.

A personalized evaluation and treatment plan will be developed for each patient, taking into account these various factors.