Angiosarcoma | Sarcoma UK
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Angiosarcoma

Angiosarcoma is a rare type of sarcoma that develops from the cells lining the blood vessels.

Angiosarcoma can occur anywhere throughout the body. But, it often occurs in the skin, breasts, internal organs, bones, and the body’s deep tissues.

In very rare cases, angiosarcoma can occur in the heart.

The cause of most angiosarcoma is unknown. But, exposure to radiation has been shown to cause some angiosarcoma. Also, swelling in the lymph nodes, known as “lymphoedema”, chemical exposure and genetic diseases are known risk factors for angiosarcoma. Researchers are still trying to learn more about the causes of angiosarcoma.

Statistics

There are an average of 164 cases of angiosarcoma diagnosed every year in England.

Angiosarcoma makes up 4.16% of all soft tissue sarcomas, and about 0.05% of all cancers.

Although angiosarcoma can affect anyone of any age, the median age at diagnosis is 71 years old.

There are slightly more females than males diagnosed with angiosarcoma.

For more information on these statistics, you can visit our soft tissue sarcoma data hub.

Signs and symptoms

Symptoms of angiosarcoma can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with angiosarcoma:

  • A lesion that looks like a bruise but doesn’t go away and grows larger
  • A lesion that bleeds when disturbed
  • A lump or swelling in the soft tissue of the body under the skin
  • This lump is often fast-growing and painful
  • Angiosarcoma is an aggressive sarcoma type and can quickly spread to other parts of the body

Diagnosis

A specialist doctor will diagnose sarcoma through a series of tests. These may include:

  • Physical examination – looking at and feeling any lump
  • A scan – taking pictures of the inside of the body using scans such as ultrasound, x-ray, CT, MRI or PET scans
  • A biopsy – taking and testing a sample of the tumour. Using this biopsy, a doctor will look to see if the biopsy looks like angiosarcoma

To read more about scans and tests, click here.

Treatment

Surgery

  • The first treatment method for angiosarcoma is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – this is known as “taking a margin”. This is to make sure that all of the cancer has been removed
  • When angiosarcoma affects the arms and legs, surgeons will do limb-sparing surgery so that the limb can continue to work well
  • Although very rare, if the cancer has spread throughout the limb, then the surgeons may have to perform a partial or full amputation to stop the cancer
  • Angiosarcomas of the breast are more likely to be found in women who have had radiotherapy to treat breast cancer. This is called “radiation-induced sarcoma”. The treatment for angiosarcoma in the breast is the removal of the breast, also known as a “mastectomy”, and chemotherapy
  • The main treatment for angiosarcoma of the heart is heart surgery. You will be operated on by a cardiovascular surgeon – a surgeon who specialises in operating on the heart and blood vessels

Radiotherapy

  • This treatment uses high-energy radiation to destroy cancer cells
  • It can be used either before or after surgery
  • When used before surgery, radiotherapy aims to make the tumour smaller so that it can be more easily removed
  • When used after surgery, radiotherapy aims to kill off any remaining cancer cells that have not been removed by surgery

Chemotherapy

  • This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back or if it has spread to other parts of the body, also known as “metastasis”

Targeted and experimental therapies

  • In angiosarcoma, researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”.

Targeted therapy: a treatment that targets specific characteristics within cancer cells in order to stop them from growing and spreading.

Immunotherapy: a treatment that helps the person’s own immune system to fight and kill cancer cells.

  • Researchers have found that some targeted therapies and immunotherapies worked well in clinical trials of angiosarcoma. But, more research and trials are needed before these treatments are approved.

After treatment

After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The follow-up will usually include:

  • A chance to discuss symptoms
  • An examination to look for any signs of angiosarcoma returning. This may include scans such as CT, MRI or ultrasound scans
  • A chest x-ray to rule out any secondary cancers occurring in the lungs

Living with

Angiosarcoma can reappear in the same area after the treatment of a previous tumour; this is called a “local recurrence”.

If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery and/or radiotherapy; your treatment will be assessed on an individual basis. It is useful to check for recurrences yourself through self-examination: your doctor or sarcoma clinical nurse specialist can tell you what to look for.

If you are worried about your cancer returning, please contact your doctor or nurse. They may decide to bring forward the date of your follow-up appointment to investigate your concerns.

What if my cancer spreads to another part of my body?

A recurrence of angiosarcoma may be accompanied by cancer in other parts of the body. This is called “metastasis” or “secondary cancer”. Some people are diagnosed with sarcoma because their metastases have been discovered before their primary angiosarcoma tumour. In angiosarcoma patients, these secondary cancers may appear in the lungs, which is why a chest x-ray is taken at follow-up appointments.

Secondary cancers may also appear in the lymph nodes, soft tissues, bone, liver and brain. Treatment for secondary cancer may involve surgery, radiotherapy or other therapy as appropriate; your treatment will be assessed on an individual basis.

Survival statistics

If you or a loved one has been diagnosed with sarcoma, you may be feeling scared and uncertain about the future. However, it’s important to remember that there is hope, and that many people with sarcoma are able to live long, fulfilling lives after their diagnosis.

Survival depends on a number of different factors. Doctors usually use large sets of data to determine the outlook for a particular type of cancer. However, sarcoma is rare, which means survival is harder to estimate than for other, more common cancers.

Here are some survival statistics for angiosarcoma:

  • 62% of people diagnosed with angiosarcoma in England survive their disease for one year or more (2013-2017).
  • 32% of people diagnosed with angiosarcoma in England survive their disease for five years or more (2013-2017).

It’s important to keep in mind that survival rates are just one factor to consider when it comes to your prognosis. Your individual prognosis will depend on many factors, including your age, overall health, and the stage and grade of your cancer.

About these statistics

The terms 1 year survival and 5 year survival don’t mean that you will only live for 1 or 5 years.

The Office for National Statistics (ONS) and researchers collect information. They watch what happens to people with cancer in the years after their diagnosis. 5 years is a common time point to measure survival. But some people live much longer than this.

5 year survival is the number of people who have not died from their cancer within 5 years after diagnosis.

If you have any questions or if you need to talk to someone, our Support Line team are here for you.

 

More information – A scientific review on the current and future management of angiosarcoma

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