Introduction to Angiosarcoma

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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.

Conclusion

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Journal of Molecular Oncology Research
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