Definition

Angiosarcoma develops from the cells that make up the walls of blood vessels. It can occur anywhere throughout the body but most commonly in the skin, breast, liver, spleen, and in the deep tissues of the body. Angiosarcoma in the skin is often found on the face and scalp. In some rare cases, angiosarcoma can occur in the heart.

Signs and symptoms

Symptoms of sarcoma 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 a sarcoma:

A bruise or a lesion that does not heal.

A lump or swelling in the in the soft tissue of the body under the skin, often on the arm, leg or trunk that is;

  • increasing in size
  • is larger than 5cm
  • usually painful, but not always

Types of diagnostic scans

The earlier sarcoma is diagnosed the better the chances of successful treatment. Sarcomas are usually found by a patient when a lump appears on the leg, arm or trunk. They can also be found during an investigation of other symptoms or during a routine operation.

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 ultrasound, x-ray, CT, EUS, PET or MRI
  • A biopsy – taking and testing a tissue sample


Types of diagnostic scans and tests

X-ray

Uses x-radiation to take images of dense tissues inside the body such as bones or tumours.

Ultrasound

A scan that uses sound waves to create images from within the body.

CT

The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area.

EUS

The Endoscopic Ultrasound Scan (EUS) uses a tube-like instrument called an endoscope with an ultrasound scanner attached. This is put inside the body to look inside the gut to investigate GIST tumours.

PET

The Positron Emission Tomography (PET) scan shows up changes in tissues that use glucose as their main source of energy – for example the brain or heart muscle. It involves an injection of a very small amount of a radioactive drug into the body. The drug travels to places where glucose is used for energy and shows up cancers because they use glucose in a different way from normal tissue.

MRI

Magnetic Resonance Imaging (MRI) uses magnets to create an image of the tissues of the body.

Histopathology

Examination of a tissue sample by a pathologist under a microscope to identify disease.

Blood test

Laboratory analysis of a blood sample.

Understanding the diagnosis

Identifying the stage and grade of a cancer means your doctor can advise on the best course of treatment for you. It also describes the cancer in a common language which is useful when your doctor is discussing your case with other doctors or healthcare professionals.

The stage of cancer is measured by how much it has grown or spread which can be seen on the results of your tests and scans. The results from a biopsy can tell what grade the cancer is.

Grading

  • Low-grade means the cancer cells are slow-growing, look quite similar to normal cells, are less aggressive, and are less likely to spread
  • Intermediate-grade means the cancer cells are growing slightly faster and look more abnormal
  • High-grade means the cancer cells are fast growing, look very abnormal, are more aggressive and are more likely to spread

Staging

  • Stage 1 means the cancer is low grade, small (less than 5cm) and has not spread to other parts of the body
  • Stage 2 means the cancer is of any grade, usually larger than stage one but has not spread to other parts of the body
  • Stage 3 means a high grade cancer that has not spread to other parts of the body
  • Stage 4 means a cancer of any grade or size that has spread to any other part of the body

Treatment

The National Institute for Health and Care Excellence (NICE) recommends that anyone with sarcoma should be referred to a specialist sarcoma team for diagnosis and treatment.

Your case will be managed by a team of experts from a wide range of health care professions called a multidisciplinary team (MDT). Your MDT will include your key worker or sarcoma clinical nurse specialist, surgeon and other healthcare professionals involved in your care. They will support you throughout your treatment to ensure you get the right treatment as and when you need it.

Types of treatment

The usual treatment for angiosarcoma is surgery. The surgeon will remove the tumour and aim to take to take an area of normal tissue around it too – this is known as taking a margin. Radiotherapy and chemotherapy may also be used to treat angiosarcoma and can be used before or after surgery. When used before surgery it aims to reduce the size of the tumour so it can be operated on and removed. When used after surgery, the aim is to kill off any local cancer cells in the area of the tumour.

Treatment for angiosarcoma of the breast

Angiosarcomas of the breast are more likely to be found in women who have previously 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 (mastectomy) and chemotherapy. Radiotherapy may not be possible if you have had radiotherapy for breast cancer in the past.

Treatment for angiosarcoma of the heart

The main treatment for angiosarcoma of the heart is surgery. You will be operated on by a cardiovascular surgeon – a surgeon who specialises in operating on the heart and blood vessels. The surgeon will remove the tumour and aim to take to take an area of normal tissue around it too – this is known as taking a margin. Unfortunately, in a lot of cases, it isn't possible to take all the tumour out. Other treatment options include chemotherapy and radiotherapy.

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 usual practice will include:

  • A chance to discuss symptoms
  • An examination to look for any signs of the sarcoma returning. This may include an MRI or ultrasound if required after examination
  • A chest x-ray to rule out any secondary cancers occurring in the lungs

Recurrence

Sarcoma cancer 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 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 sarcoma 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 sarcoma tumour. In sarcoma 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 liver or brain. Treatment for secondary cancer may involve surgery, radiotherapy or chemotherapy as appropriate; your treatment will be assessed on an individual basis.