Angiosarcoma

What is angiosarcoma?

Angiosarcoma is a very rare cancerous tumor that starts in the inner lining of your blood vessels or lymphatic vessels. Because these tumors arise from vessels located throughout your body, you can develop angiosarcoma almost anywhere.

That said, angiosarcoma usually affects the skin on your head, neck or breast (cutaneous angiosarcoma). Less often, it starts in organs like your heart (cardiac angiosarcoma), liver and spleen.

Learning you have angiosarcoma can feel unsettling because it can be an aggressive cancer that’s hard to treat. But that doesn’t mean you don’t have options. Your healthcare provider can advise you on treatments based on your diagnosis.

How common is this condition?

Angiosarcomas are rare malignant soft tissue sarcomas, which are rare tumors in and of themselves. Each year, approximately 1 person in 1 million people in the U.S. is diagnosed with angiosarcoma. The most common site where you get angiosarcoma is your skin, especially your scalp.

What are the symptoms of angiosarcoma?

Angiosarcoma symptoms vary depending on the area of your body affected. For example, angiosarcoma of your skin (including your scalp, face, neck and breast) may look like:

• Reddish or blue small lumps that eventually spread, grow bigger and bleed easily.
• A purplish area of skin that looks like a rash or bruise (may be raised).
• A sore that doesn’t heal or that seems to grow.

You may not notice symptoms when angiosarcoma is in an organ, like your liver, until the tumor grows big enough to impact how the organ works. Or the angiosarcoma may create pressure on nearby structures. Symptoms of angiosarcoma in an organ may include:

• Fatigue.
• Pain near the affected area.
• Malaise.
• Unexplained weight loss.
• Shortness of breath (most common symptom of cardiac angiosarcoma).
• Jaundice and persistent pain in the upper left part of your belly (possible symptoms of hepatic angiosarcoma).

How painful is angiosarcoma?

An angiosarcoma may or may not cause pain. For example, angiosarcomas in your liver and spleen may cause pain in your upper abdomen, but usually not until they’ve gotten big and become more advanced. Angiosarcomas on your skin may not hurt at all. Instead, they may look like a bruise that doesn’t fade or spreads.

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 cancerous cells don’t die. Instead, they continue to make more cells that eventually become tumors. The cancerous cells keep on growing from your affected blood vessels. Often, they spread to other areas of your body.

Risk factors

Researchers have identified several risk factors that may increase the chance you’ll develop angiosarcoma. These risk factors are:

• Age and sex: While angiosarcoma can affect anyone at any age, people aged 60 and older are more likely to develop angiosarcoma. More men and people assigned male at birth (AMAB) than women and people assigned female at birth (AFAB) develop primary angiosarcoma.
• Radiation therapy: Angiosarcoma is sometimes a secondary cancer that people develop several years (usually around eight to 10) after getting radiation treatment for a different cancer type, most commonly breast cancer.
• Exposure to chemicals: Liver (hepatic) angiosarcoma is associated with exposure to polyvinyl chloride, arsenic and thorium dioxide. Sometimes, angiosarcomas don’t show up until 10 to 40 years after exposure.
• Chronic lymphedema: Approximately 5% of all angiosarcomas are linked to a form of chronic (long-term) lymphedema called Stewart-Treves syndrome. It most often affects people who had a mastectomy and lymph node removal during breast cancer treatment.
• Genetic disorders: About 3% of all angiosarcomas affect people who have conditions caused by genetic disorders. Specific conditions include bilateral retinoblastoma, Maffucci syndrome, neurofibromatosis and Klippel-Trenaunay syndrome.

How is angiosarcoma diagnosed?

A healthcare provider may start by doing a physical exam and discussing your symptoms. They’ll also ask about your medical history. For example, previous radiation therapy or working a job that exposed you to large amounts of certain chemicals may cause your provider to suspect angiosarcoma.

They may do imaging tests to look at the tumor’s size and location. A CT scan, MRI and PET scan are the most common tests used to diagnose angiosarcoma. You may need a mammogram or ultrasound if your provider suspects a breast angiosarcoma. You may need a transesophageal echocardiogram if your provider suspects a cardiac angiosarcoma.

Finally, you’ll need a biopsy, which involves a provider removing small samples of your tissue, fluid and cells. The samples are sent to a laboratory so a pathologist can test for cancer cells. A lab test called immunohistochemistry can confirm an angiosarcoma diagnosis.

Stages of angiosarcoma

Cancer staging helps healthcare providers classify how advanced cancer is. Providers stage angiosarcomas on a scale from I to IV, with Stage I meaning the tumor hasn’t spread beyond its primary location and Stage IV meaning it’s spread to distant parts of the body. As angiosarcomas are aggressive, many have already spread (which means they’re more advanced) by the time they’re diagnosed. The most common place they spread is to the lungs.

Knowing your cancer stage can help your provider decide which treatments may help you live longer.

How are angiosarcomas treated?

Surgery to remove the tumor is the most common treatment for angiosarcoma. Healthcare providers may recommend radiation therapy or chemotherapy before or after surgery to shrink tumors or kill additional cancer cells.

Newer cancer treatments, like targeted therapy drugs and immunotherapy, are currently in development to fight angiosarcoma. Depending on your situation, your provider may recommend one of these treatments or a clinical trial to test new cancer-fighting therapies.

Can angiosarcoma be prevented?

Not all causes of angiosarcoma are preventable. For example, you can’t help inheriting a genetic condition or needing radiation therapy for cancer. But there are things you can do to reduce some risks of developing angiosarcoma, like limiting your exposure to certain toxic chemicals.

What can I expect if I have angiosarcoma?

Angiosarcomas can spread fast because they start in your blood vessels. Once it’s spread, angiosarcoma is very difficult to treat. Even in those cases where it goes away after treatment, it often comes back (recurs).

Healthcare providers are identifying more effective ways of treating angiosarcoma, but the survival rate is still low. About 35% of people diagnosed with angiosarcoma are alive five years after diagnosis.

How do I take care of myself?

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:

• Try to ease your stress. If you’re feeling extra stressed after learning of your diagnosis, you’re not alone. Cancer is stressful. You may find activities like meditation, relaxation exercises or deep breathing help.
• Allow yourself to rest. You may feel very tired or “wiped out” after your cancer treatments. Try to get as much rest as you can.
• Try to eat healthy foods regularly. Your treatments might affect your appetite. Try to eat nutritious foods (without skipping meals). Talk to a dietitian if you’re having trouble eating.
• Connect with others. Cancer can be lonely, especially when you’re dealing with a rare cancer like angiosarcoma. Ask your provider to connect you to support groups where you can share your feelings with people who understand what you’re going through.

When should I see my healthcare provider?

Schedule a visit with a healthcare provider if you notice any changes to your skin. This includes nodules or sores on your scalp, face or neck that aren’t getting better. Reach out if you’re noticing new bruising in an area where you had prior radiation therapy.

If you’re already receiving angiosarcoma treatment, contact your provider if your symptoms seem to be getting worse. Let them know if 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 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 have persistent diarrhea.

What questions should I ask my healthcare provider?

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 stage is my angiosarcoma?
• What treatments do you recommend?
• What are the side effects?
• How likely is it that the cancer will come back after treatment?
• Should I participate in a clinical trial?
• What’s my prognosis?