Angiosarcoma is a form of cancer that develops in the inner lining of the blood and lymph vessels. This cancer affects both adults and children; however, we do not see this cancer as often in young patients.
Angiosarcoma accounts for two percent of cancers. These tumors usually grow under the skin in adults, and in soft tissue or organs in children.
Angiosarcoma is not like other common tumors that develop in blood vessels, since it can spread to other parts of the body.
What Causes Angiosarcoma?
Angiosarcoma sometimes run in families and is linked to a gene called POTI.
Risk factors include:
- Lymphedema (when extra fluid builds up)
- Previous radiation
- Exposure to chemicals
What Are the Signs and Symptoms of Angiosarcoma?
A common sign of angiosarcoma is a painless lump or mass in soft tissue of the body. This mass can be large or small, and can grow in size over time. The mass may be found under the skin, or deep in the muscles or other tissues.
“Cutaneous angiosarcoma” may first appear like a bruise and can bleed easily if scratched or bumped. Very rarely, infants with angiosarcoma may have many tumors of the skin and liver at the same time.
Angiosarcoma can be present in the body for months or even years before a patient notices a lump.
How Is Angiosarcoma Diagnosed?
A physical exam and imaging tests help the doctor diagnose angiosarcoma. These tests allow the doctor to see where the tumor is inside the body, its size, and if it has spread to other parts of the body.
Imaging tests might include:
- Magnetic resonance imaging (MRI)
- Body computed tomography (CT)
- A chest CT
- Positron emission tomography (PET), a test that can help show if the cancer has spread.
Patients will also have a procedure (called a needle biopsy) where the doctor removes a small piece of the tumor. A specialist examines the tissue under a microscope to check for angiosarcoma.
What Are the Stages of Angiosarcoma?
Staging of angiosarcoma is based on the size of the tumor and whether it has spread to lymph nodes or distant sites in the body. If the tumor has not spread, it is considered to be localized. Metastatic angiosarcoma means the tumor has spread past nearby tissues to lungs or other organs.
How Is Angiosarcoma Treated?
The patient may have a procedure, called a wide resection with margins, to remove the tumor and some tissue around the tumor. If the doctor is concerned that the cancer has also spread to nearby lymph nodes, they may also be removed.
Patients may have radiation treatment before surgery to make the tumor smaller. After surgery, additional radiation therapy can help kill any tumor cells left in the body.
The doctor will make a plan for radiation based on the treatment goals and the location of the tumor.
Two types of radiation are used, regular radiation or proton radiation. Proton radiation has less side effects because it kills the tumor but does not hurt organs and healthy tissue. Cincinnati Children’s is one of the few pediatric hospitals in the country that offers proton therapy.
If the tumor is large or has spread to other areas, the doctor may recommend chemotherapy or other types of drugs to help kill the cancer cells.
Clinical Research Trials
Angiosarcoma can be challenging to treat. In some situations, the doctor may recommend participating in a clinical trial at Cincinnati Children's.
Follow-up imaging tests can help monitor how the cancer is responding to treatment.
What Is the Long-Term Outlook for People with Angiosarcoma?
Many patients with angiosarcoma can be cured with surgery alone. However, angiosarcoma that has spread (metastasized) is hard to cure. Researchers are working to better understand angiosarcoma and find more effective treatment options.