Angiosarcoma

Angiosarcomas are a rare, fast-growing type of soft tissue sarcoma. These cancerous tumors form in the inner lining of your blood vessels or lymphatic vessels. You have these vessels throughout your body. That’s why these tumors can quickly spread from where they started.

Healthcare providers may say you have a primary or metastatic angiosarcoma. A primary tumor hasn’t spread from where it started. A metastatic tumor is when cancer spreads to another part of your body. This condition typically affects your skin, but it can develop in organs like your heart and liver.

Treatment includes surgery along with chemotherapy and radiation therapy. Angiosarcomas (pronounced "an-jee-oh-sar-KOH-muhs") often come back. This condition can develop almost anywhere in your body. The most common site is your skin.

Angiosarcoma types

This condition is classified by where it starts in your body. The different types are:

• Angiosarcoma of the breast: This condition is a tumor in your breast (primary angiosarcoma of the breast). It may affect your breast skin and spread to your bones, liver or lungs.
• Cutaneous angiosarcoma: This type typically affects the skin on your head or neck but can happen anywhere on your body. It may spread to your lungs, liver or lymph nodes.
• Cardiac angiosarcoma: This means there are tumors in your heart’s blood vessels and muscle. The tumors typically spread to your lungs, but they may also affect your brain, liver, bones, spleen, skin and spine.
• Liver (hepatic) angiosarcoma: This type starts in your liver’s blood vessels. It’s a very rare form of primary liver cancer. It can spread to your lungs, lymph nodes, spleen and bones.

Symptoms of angiosarcoma

Angiosarcoma symptoms vary depending on the area of your body the condition affects. For example, a cutaneous angiosarcoma may cause symptoms like:

• Small reddish or blue lumps on your neck, head or breast that may grow or bleed
• A purple rash or bruise that doesn’t go away and may be raised
• A sore that doesn’t heal or gets bigger

An angiosarcoma in places like your heart or liver may not cause symptoms until the tumor grows large enough to affect how an organ works. Some symptoms include:

• Fatigue
• Losing weight without trying
• Pain in the upper left part of your belly (from an angiosarcoma in your liver)
• Shortness of breath (common with cardiac angiosarcoma)

Angiosarcoma causes

This condition happens when cells in the inner lining of your blood vessels or lymphatic vessels become cancerous and form tumors. Experts don’t know why this happens. But certain factors increase your risk. These include:

• Age and sex: Males age 60 and older are most likely to have this condition.
• Chronic lymphedema: People who have long-term lymphedema after lymph node removal or mastectomy may develop angiosarcoma.
• Exposure to chemicals: Liver angiosarcoma is associated with exposure to polyvinyl chloride, arsenic and thorium dioxide.
• Exposure to sunlight: Long-term exposure to sunlight may increase your risk of cutaneous (skin) angiosarcoma.
• Genetic conditions: People with bilateral retinoblastoma, Maffucci syndrome, neurofibromatosis type 1 or Klippel-Trenaunay syndrome have an increased risk.
• Radiation therapy: People who have had radiation therapy, most often for breast cancer, may develop angiosarcoma.

How doctors diagnose angiosarcoma

A healthcare provider may start by doing a physical exam and discussing your symptoms. They’ll also ask about your medical history. For example, they may ask if you’ve been exposed to certain chemicals or received radiation therapy for breast cancer. They may do tests, including:

• Imaging tests, like CT, MRI or PET scans, to check the tumor’s size and location
• Mammogram or breast ultrasound to check for tumors in your breast
• Transesophageal echocardiogram (TEE) to check your heart’s blood vessels and muscle
• Biopsy to get tissue samples for analysis under a microscope

Angiosarcoma staging

Cancer staging helps healthcare providers plan treatment. Providers stage angiosarcomas on a scale from 1 (I) to 4 (IV). Stage 1 (I) means the cancer hasn’t spread from where it started. Providers may call this localized cancer. Stage 4 (IV) is cancer that’s spread to more distant tissues and organs.

How are angiosarcomas treated?

Surgery to remove the tumor is the most common treatment. Healthcare providers may recommend radiation therapy or chemotherapy to shrink the tumors before surgery. You may have more treatment after surgery to kill any cancer cells that remain.

Researchers are testing treatments like targeted therapy and immunotherapy to see if they’re effective against angiosarcoma.

When should I see my healthcare provider?

Cutaneous angiosarcoma is the most common form of this rare condition. Talk to a healthcare provider if you have changes in your skin, like:

• Bruises that don’t fade
• Sores that don’t heal
• Lumps that get bigger

If you’re already receiving angiosarcoma treatment, contact your provider if your symptoms don’t improve. And let your provider know about changes in your body that may be new symptoms.

Ask your provider for help if you have an unexpected or unusually strong reaction to treatment. They can take steps, like changing your medication or reducing the dosage, to ease your symptoms.

Can angiosarcoma be cured?

Treatment can help you live longer with this condition. But it often comes back.

What’s the survival rate?

One study found that, overall, 27 out of 100 people were alive five years after diagnosis. It’s important to remember that there are different types of angiosarcoma. What you can expect depends on factors like the condition type and stage at diagnosis. Survival rate data can be confusing. Ask your healthcare provider to explain what a survival rate means in your case.