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Angiosarcoma

To view this article in the new Rare Cancers Australia Knowledgebase, click here 

What is Angiosarcoma?

Angiosarcoma is a cancer of the inner lining of blood vessels, and it can occur in any area of the body. The disease most commonly occurs in the skin, breast, liver, spleen, and deep tissue. Angiosarcoma of the skin, or cutaneous angiosarcoma, makes up the majority of angiosarcoma cases, and it is usually found on the scalp and face. Angiosarcoma that appears underneath the surface of the skin is called subcutaneous angiosarcoma. Approximately 25% of angiosarcoma are found in deep tissue, and around 8% are found in breast tissue. 

There are 2 main types. Haemangiosarcomas start in blood vessel walls and lymphangiosarcomas start in lymph vessel walls. But doctors don't tend to refer to these different types, and generally just use the term angiosarcoma. 

Breast angiosarcoma are very rare and it is difficult to find much information about them. Medical journals and textbooks contain very few reports of primary breast angiosarcoma. Most reported cases are in younger women. They are more likely in women who have already had treatment for breast cancer with surgery and radiotherapy, some years previously. They tend to grow very quickly and are generally difficult to successfully treat.

Angiosarcomas occur in men and women of all races, and they are rare in children. Patients with angiosarcoma are best treated at a cancer centre where an expert sarcoma team and resources are available to provide specialized and responsive care.

Vascular Sarcoma

Vascular sarcomas develop from cells that make up the walls of blood or lymphatic vessels. Around 145 people are diagnosed with this in the UK every year.

There are different types of vascular sarcoma. Angiosarcomas start from the cells that make up the walls of blood or lymphatic vessels. If they start in the blood vessels they are called haemangiosarcomas. If they start in the lymph vessels they are called lymphangiosarcomas.

Another type of vascular sarcoma is called haemangioendothelioma. This tumour may start in soft tissues or in internal organs, such as the liver or lungs. More on hemangioendothelioma can be found here.

Alveolar soft part sarcoma (ASPS) 

Alveolar soft part sarcoma is a rare, slow growing and highly angiogenic (vessel forming) sarcoma (malignant tumor of connective tissue) of an unclear cause. It is among the least common sarcomas, representing 0.02-0.10% of large studies of soft tissue sarcomas. 
 
It is characterized by a painless mass in the leg or buttock, with a particular affinity to travel to the lungs as multiple nodules, presumably while the sarcoma itself is still small. ASPS, is very rare, because it involves a specific breaking and joining event between two chromosomes, called an "unbalanced translocation".  

Signs and Symptoms 

  • Painless thigh or buttock mass 
  • Limping or other difficulty with movement 
  • Cough, sharp chest pain, pleural effusions can all occur in advanced disease  

Causes 

No exposure or infection that is known to predispose to ASPS. It is known that two chromosomes break and rejoin is a certain way (unbalanced translocation) and bring together two genes, normally separated on chromosomes X (the sex chromosome) and 17. These genes are called ASPL on chromosome 17 and TFE3 on chromosome X.  
 
ASPS, tends to affect younger people, between 15 and 35 years of age. Women outnumber men, especially under age 25. There appears to be no link of this tumor to a particular ethnicity. 

What causes angiosarcoma? 

In most cases, the cause of a sarcoma is unknown. The most widely known cause of angiosarcoma is lymphedema, the swelling of an area of the body due to the collection of fluid. Angiosarcoma can also occur due to radiation exposure or treatment, and angiosarcoma has been associated with carcinogens such as vinyl chloride, arsenic and thorium dioxide.

Symptoms of angiosarcoma

Angiosarcoma can present in many different ways. It can look like a skin infection, a bruise or a lesion that does not heal. It may have a violet colour, and one should be particularly concerned if such an area arises in a site of prior radiation therapy. It might also present as a soft lump that can be felt or seen. Deep-seated tumours may go unnoticed until they begin to affect surrounding tissues and organs.

  • Angiosarcoma of the breast symptoms: 
    • A lump or thickening in an area of the breast
    • A change in the size or shape of a breast
    • Dimpling of the skin
    • A change in the shape of your nipple, particularly if it turns in, sinks into the breast, or has an irregular shape
    • A blood stained discharge from the nipple
    • A rash on a nipple or surrounding area
    • A swelling or lump in the armpit

How is angiosarcoma diagnosed?

Your GP usually refers you to a specialist breast clinic. At the breast clinic the doctor or specialist nurse takes your medical history and examines your breasts. They also feel for any enlarged lymph nodes under your arms and at the base of your neck. You will have some of the following tests:

  • A physical exam
  • MRI or PET scan
  • CT scan 
  • A mammogram (an X-ray of the breasts)
  • An ultrasound (if you are under 35 you are more likely to have an ultrasound scan instead of a mammogram)
  • A biopsy – a small sample of cells or tissue is taken and looked at under a microscope

A definite diagnosis is made with a biopsy procedure, where a small piece of the tumour is removed from the body and looked at under a microscope by a pathologist. Most angiosarcoma are high grade tumours that are aggressive and fast-growing, but some are low-grade tumours that are less aggressive and slow-growing. 

How is angiosarcoma treated? 

Surgery is the primary method of treatment for angiosarcoma. Chemotherapy and/or radiation therapy can be an important part of the treatment plan, and they may be administered before or after surgery.  The chemotherapy of choice for angiosarcoma has been doxorubicin, and some centres use a combination of mesna, doxorubicin, and ifosfamide (MAI). Liposomal Doxorubicin has also been utilized. Paclitaxel and Docetaxel have shown effectiveness against angiosarcoma of the head, neck and scalp.

Because breast angiosarcoma are so rare, there is no established standard treatment regime. Removal of the breast (mastectomy) and chemotherapy are the most likely choices of treatment. The chemotherapy drugs may be different from those usually used to treat other types of breast cancer.

What is the prognosis for angiosarcoma patients?

Unfortunately, many angiosarcoma patients are diagnosed after the disease has spread throughout the body, and this late diagnosis results in a poor prognosis. Studies suggest that better outcomes are achieved for patients who have smaller tumours that are removed with clear margins. Low grade angiosarcoma of the breast is also reported as having a better prognosis. 

Angiosarcoma patients should be closely monitored after treatment. A suggested schedule of follow-up includes an appointment every three months for the first two years, then every six months until the five-year mark.

For more information on Angiosarcoma, click here

Information has also been sourced from rarediseases.org, curesarcoma.org, and cancerresearchuk.org

For clinical trials that may be available for this cancer in Australia, click here 

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