What is a Lymphangiosarcoma?

Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities.

What does angiosarcoma mean?

Angiosarcoma is a rare cancer that develops in the inner lining of blood vessels and lymph vessels. This cancer can occur anywhere in the body but most often is in the skin, breast, liver and spleen.

What other issues can Lymphangiosarcoma cause?

These include congenital lymphedema (Milroy’s disease), lymphedema following other types of surgery, and chronic venous stasis. Lymphedematous tissue is immunologically vulnerable for the development of infections and neoplasms.

Is Lymphangiosarcoma malignant?

Lymphangiosarcoma is a malignant neoplasm derived from endothelial cells of lymphatic vessels.

How common is lymphangiosarcoma?

Lymphangiosarcoma following breast cancer is a rare entity. Around 300 cases have been reported worldwide since Stewart-Traves syndrome characterized by angio sarcoma of skin and soft tissue of the area after Radical Mastectomy, Radiation and Lymphedema [1].

What causes angiosarcoma?

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 is the difference between sarcoma and angiosarcoma?

Angiosarcoma is a form of soft tissue sarcoma. It is a rare cancer in which the cells that make up the lining of the blood vessels grow and multiply abnormally to form a tumor. Angiosarcoma is more common in people over 50.

What are the symptoms of lymphangiosarcoma?

Symptoms of lymphangiosarcoma may include the following:

• Chronic swelling of the affected area.
• Painful nodules in the area of swelling.
• Non-healing painful ulcers.
• Bleeding and oozing from the ulcers.

How do you get lymphangiosarcoma?

Lymphangiosarcoma usually develops in patients with a history of chronic lymphedema, after mastectomy for breast cancer. If left untreated, chronic lymphedema leads to severe chronic edema of the affected extremity. The skin becomes atrophic and pachydermatous with telangiectasias.

How can you prevent lymphangiosarcoma?

The most efficient way to avoid Stewart-Treves syndrome, a highly lethal disease, is by preventing or treating long-standing lymphedema, which predisposes individuals to this lymphangiosarcoma years later.