Angiosarcoma

What is angiosarcoma?

Angiosarcoma is a very rare soft tissue tumor that affects the inner lining of your blood vessels. Because angiosarcoma arises from your blood vessels, you can develop an angiosarcoma almost anywhere in your body. That said, angiosarcoma most commonly affects your skin, breasts, liver or head and neck.

Are angiosarcomas common?

Angiosarcomas are malignant soft tissue sarcomas, which are rare tumors in and of themselves. Soft tissue sarcomas represent 1% of all solid malignancies or tumors, and angiosarcomas represent about 1% of all soft tissue sarcomas. Each year, approximately 1 person in 1 million people in the United States is diagnosed with angiosarcoma. Cutaneous angiosarcoma, which is skin cancer, is the most common form of angiosarcoma.

Who’s affected by angiosarcoma?

While angiosarcoma can affect anyone at any age, people aged 60 and older are more likely to develop angiosarcoma, and more people who are assigned male at birth (AMAB) than people who are assigned female at birth (AFAB) develop primary angiosarcoma.

What causes angiosarcoma?

Angiosarcoma happens when the cells in the inner lining of your blood vessels change from normal to abnormal cells. Unlike normal cells, the abnormal cells don’t die but continue to churn out abnormal cells that eventually become masses or tumors. The abnormal cells keep on growing from your affected blood vessels, sometimes spreading to other areas of your body.

Researchers believe these tumors start forming when something changes in your blood vessel and/or lymph vessels’ genetic code. They’ve also identified several risk factors that may increase the chance you’ll develop angiosarcoma. Those risk factors are:

• Having radiation therapy, particularly if you received radiation therapy for breast cancer. Researchers believe there’s a link between radiation therapy and angiosarcoma that can develop many years after you’ve received radiation therapy.
• Being exposed to chemicals, including polyvinyl chloride, arsenic and thorium dioxide.
• Having chronic lymphedema. Approximately 5% of all angiosarcomas are linked to a form of chronic lymphedema called Stewart-Treves syndrome.
• Familial syndromes. About 3% of all angiosarcomas affect people who have conditions caused by genetic disorders.

Genetic disorders linked to angiosarcoma

Researchers have found links between genetic disorders that affect genes that are supposed to protect you against tumors. Specific conditions include:

• Bilateral retinoblastoma. This rare cancer grows on your retina on the back of your eyes.
• Ollier disease. This rare disorder affects how your bones develop.
• Maffucci syndrome. This rare disorder affects your bones’ cartilage.

What are angiosarcoma symptoms?

Angiosarcoma symptoms vary depending on the area of your body affected by angiosarcoma. For example, symptoms of angiosarcoma of your skin include reddish or blue nodules on your scalp, head and neck that bleed easily. Other examples are:

• Angiosarcoma of your liver. Symptoms include jaundice, fatigue and persistent pain in the upper left part of your belly.
• Angiosarcoma of your breast. Symptoms may include skin changes such as purple-colored nodules on your breast or a lump in your breast.

How do healthcare providers diagnose angiosarcoma?

Healthcare providers may start by discussing your medical history and your symptoms. They may do imaging tests to look at the tumor’s size and location. They may also do a biopsy, removing small samples of your tissue, fluid and cells. The samples are sent to a laboratory for examination under a microscope.

How do healthcare providers treat angiosarcomas?

Surgery to remove your tumor is the most common treatment for angiosarcoma. Healthcare providers may recommend radiation therapy or chemotherapy before or after surgery. This is adjuvant therapy. Each treatment will vary depending on your specific situation and factors such as your age and your overall health. Your healthcare provider will explain each treatment and potential side effects.

How can I reduce my risk of developing angiosarcoma?

Researchers have identified several activities and medical conditions that might increase your risks for developing angiosarcoma. For example, you may reduce your risk by protecting your skin from the sun and limiting your exposure to certain chemicals. Some risk factors you can’t avoid, such as inheriting a genetic condition or needing radiation therapy for cancer.

What should I do if I think I’m at risk for angiosarcoma?

Like most cancers, early diagnosis and treatment improve your expected outcome. Tell your healthcare provider about your specific situation so they can assess your risk. They may recommend ways to monitor your health for signs of angiosarcoma.

What can I expect if I have angiosarcoma?

Angiosarcomas can spread very quickly because they start in your blood vessels. Once it’s spread, angiosarcoma is very difficult to treat. Even though healthcare providers have more effective ways of treating angiosarcoma, about 35% of people diagnosed with angiosarcoma are alive five years after diagnosis.

How do I take care of myself if I have angiosarcoma?

Angiosarcoma is a fast-moving cancer. You can take care of yourself by slowing down. Try to give yourself time to understand what’s happening to your body. Talk to your healthcare provider about steps you can take to support your treatment. Here are some suggested steps that may help:

• Cancer is stressful. You might find activities such as meditation, relaxation exercises or deep breathing help to ease your stress.
• Your treatments might affect your appetite. Try to eat a healthy diet, and talk to a nutritionist if you’re having trouble eating.
• You may feel very tired or “wiped out” after your cancer treatments. Try to get as much rest as you can.
• Cancer can be lonely. Sometimes, it’s hard talking to loved ones about your condition. Your healthcare provider can direct you to support groups and programs where you can share your thoughts and feelings with people who understand what you’re going through.

When should I see my healthcare provider?

Your healthcare provider will talk to you about your symptoms and treatment side effects. You should contact your healthcare provider if your angiosarcoma symptoms seem to be getting worse or you have an unexpected or unusually strong reaction to treatment.

When should I go to the emergency room?

Some cancer treatments affect your immune system and increase your risk of developing infections. If you’ve had surgery, you may have problems with your surgery site. You should go to the emergency room if:

• You have a fever that’s higher than 100.4 degrees Fahrenheit (38.3 degrees Celsius). A fever may be a sign you have an infection.
• You have pain that your pain medication doesn’t help.
• You’re vomiting a lot or you have persistent diarrhea.

What questions should I ask my doctor?

Angiosarcoma is a very rare cancer. You may wonder why you developed this cancer and what your healthcare provider can do to help you. Here are some suggestions for questions to help you with that conversation:

• What is angiosarcoma?
• Do I have cancer in more than one place in my body?
• What treatments do you recommend?
• What are the side effects?
• Will my cancer come back after treatment?
• Should I consider participating in a clinical trial?
• What’s my prognosis?

A note from Cleveland Clinic

Angiosarcoma is a very rare cancer that spreads very quickly. If you have angiosarcoma, you may be dealing with advanced cancer. You may feel overwhelmed by your situation and worried about what’ll happen next. Your healthcare provider knows what you’re going through. Share your concerns and feeling with them so they can recommend programs and services that may help you.