Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are more commonly affected, and the skin is the most affected area, with approximately 60% of cases being cutaneous (skin). Specifically, the scalp makes up ~50% of angiosarcoma cases, but this is still <0.1% of all head and neck tumors. Infrequently they have occurred in association with breast implants. Ultraviolet radiation This makes them especially lethal, and an early diagnosis is usually necessary for survival. Even with treatment, prognosis is poor, with a five-year survival rate of 30–38%.
Primary cutaneous angiosarcoma
Angiosarcoma in the skin (usually the head or neck and specifically the scalp) with no underlying risk factors such as lymphedema or radiation therapy.
Lymphedema associated angiosarcoma
Also called Stewart-Treves syndrome, it is a lymphangiosarcoma resulting from chronic lymphedema, swelling due to the build up of lymphatic fluid. It typically happens in the breast following a mastectomy that removes the lymph nodes of the breast. However it can happen anywhere.
Primary cardiac angiosarcoma
Primary cardiac angiosarcoma is an angiosarcoma that arises in the heart. Despite being very rare they are the most common malignant primary heart tumor with 10–25% The underlying causes of DNA damage is not understood but several risk factors are widely accepted.
Accepted risk factors include lymphedema, radiation therapy, neurofibromatosis, Maffucci syndrome, Klippel-Trenaunay syndrome
Immunosuppression may play a role in the pathogenesis of angiosarcoma. There have been reports of angiosarcoma in immunosuppressed individuals following kidney transplantation. The association between lymphedema and angiosarcoma may be due to localized immunodeficiency, however this hypothesis has not been confirmed. Some studies suggest a link between AIDS and angiosarcoma though this may be due to misdiagnosed Kaposi's sarcoma. and cutaneous angiosarcoma where males are again affected more commonly, particularly elderly white men.
See also
- Glomangiosarcoma
- List of cutaneous conditions