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 angiosarcomas are found in deep
tissue, and around 8% are found in breast tissue.

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

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.

What are the 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
color, 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 tumors may go unnoticed until they
begin to affect surrounding tissues and organs.

How is angiosarcoma diagnosed?

Diagnosis usually begins with a physical exam and may include imaging
studies such as an X-ray, CT scan, MRI or PET scan. These studies help to
determine the location and size of the tumor and can also find disease
that has spread throughout the body.

A definite diagnosis is made with a biopsy procedure, where a small

piece of the tumor is removed from the body and looked at under a
microscope by a pathologist. Most angiosarcomas are high grade tumors
that are aggressive and fast-growing, but some are low-grade tumors
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 angiosarcomas has been doxorubicin,
and some centers use a combination of mesna, doxorubicin, and
ifosfamide (MAI). Liposomal Doxorubicin has also been utilized. Paclitaxel
and Docetaxel have shown effectiveness against angiosarcomas of the
head, neck and scalp.

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 tumors that are removed with clear margins.
Low grade angiosarcoma of the breast is also reported as having a better
prognosis.

Follow-Up
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