Angiosarcoma

What causes angiosarcoma?

It's not clear what causes most angiosarcomas, though doctors have identified factors that may increase your risk of the disease.

Doctors know that something happens that causes a cell in the lining of a blood vessel or lymph vessel to develop an error (mutation) in its genetic code. The mutation tells the cell to grow quickly, making more abnormal cells. The abnormal cells continue living when other cells would die.

The result is a buildup of abnormal cells that grows from the affected blood vessel or lymph vessel. With time, cells may break off and spread (metastasize) to other areas of the body.

Risk factors

Factors that may increase your risk of angiosarcoma include:

  • Radiation therapy. Treatment with radiation for cancer or other conditions may increase your risk of angiosarcoma. A rare complication of radiation therapy, angiosarcoma typically occurs five to 10 years after treatment.
  • Swelling caused by lymph vessel damage (lymphedema). Lymphedema is swelling caused by a backup of lymph fluid that occurs when the lymphatic system is blocked or damaged. Lymphedema is a risk whenever lymph nodes are removed during surgery — a technique that's often used to treat cancer. Lymphedema can also occur in response to infection or other conditions.
  • Chemicals. Liver angiosarcoma has been linked to exposure to several chemicals, including vinyl chloride and arsenic.

Last updated on 05-01-20

How is angiosarcoma diagnosed?

Tests and procedures used in angiosarcoma diagnosis include:

  • Physical exam. Your doctor will thoroughly examine you to understand your condition.
  • Removing a sample of tissue for testing (biopsy). Your doctor will remove a sample of suspicious tissue for laboratory testing. Analysis in the lab can detect cancer cells and determine certain characteristics of your cancer cells that may help guide your treatment.
  • Imaging tests. Imaging tests can give your doctor an idea of the extent of your cancer. Tests may include MRI, CT and positron emission tomography (PET). Which tests you undergo will depend on your particular situation.

Last updated on 05-01-20

How is angiosarcoma treated?

Which angiosarcoma treatment is best for you depends on your cancer's location, its size and whether it has spread to other areas of your body.

Treatment options may include:

  • Surgery. The goal of surgery is to remove the angiosarcoma entirely. Your surgeon will remove the cancer and some of the healthy tissue that surrounds it. In some cases surgery may not be an option, for example, if the cancer is very large or has spread to other areas of the body.
  • Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells. Radiation therapy is sometimes used after surgery to kill any cancer cells that remain. Radiation therapy may also be an option if you can't undergo surgery.
  • Chemotherapy. Chemotherapy is a treatment that uses drugs or chemicals to kill cancer cells. Chemotherapy may be an option if your angiosarcoma has spread to other areas of your body. In certain situations, it may be combined with radiation therapy if you can't undergo surgery.

Last updated on 05-01-20

Spiker AM, Ramsey ML. Cancer, Angiosarcoma StatPearls. Updated Jan 24, 2019; Reference Link

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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