Sarcoma is a type of cancer that develops in connective tissues like bone, cartilage, fat, blood vessels, muscle or fibrous tissues (related to tendons or ligaments).
The disease usually causes tumors to form in the tissues below the skin or face or in the mucous membranes of the nose, mouth or anus. Patients typically suffer from lesions on the skin, which appear as raised blotches or lumps that may be purple, brown or red.
While the skin lesions may be disfiguring, they are not usually life threatening. In some cases, the lesions may be painful or cause swelling. If KS develops in the lungs, liver or gastrointestinal tract, however, the disease may be life threatening, causing internal bleeding or difficulty breathing.
Kaposi's sarcoma (KS) was named after Dr. Moritz Kaposi who first described the disease in 1872. For decades KS was considered a rare disease that primarily affected elderly men of Mediterranean or Jewish descent, organ transplant recipients or young adult African men. This type is called classic KS.
However, in the last 20 years, most KS cases have developed in association with the human immunodeficiency virus (HIV) and the acquired immune deficiency syndrome (AIDS). This type is called HIV-related KS, AIDS-related KS or epidemic KS.
Researchers believe a herpes virus called human herpes virus 8 (HHV-8) causes HIV-related Kaposi's sarcoma. In a recent study, men who had HHV-8 were nearly 12 times more likely to develop KS than men who did not have HHV-8. The herpes virus is usually dormant in healthy individuals. However, immunocompromised patients, including HIV/AIDS patients, may develop KS as a result of the infection.
According to the U.S. Centers for Disease Control and Prevention (CDC), KS is considered an AIDS-defining disease. This means that when HIV-infected patients develop KS, their condition has progressed to AIDS. In the United States, KS serves as an AIDS-defining illness in about 10-15% of HIV-infected homosexual men.
With new treatments for AIDS, including antiretroviral therapy (ART), as well as greater awareness of HIV, the number of KS cases due to HIV infection has decreased about 85% to 90%, according to researchers. Before ART was available, an estimated 90% of patients who had AIDS-related KS died from the disease. Today, researchers estimate that in areas where antiretroviral therapy (HIV/AIDS treatment) is available, 47% of patients who have AIDS-related KS die.
Local therapies, including radiation therapy, intralesional vinblastine, cryotherapy (use of cold temperatures to treat disease) and retinoids have been used to treat patients who experience localized lesions. However, local therapy does not stop the development of new lesions. Systemic therapies, including interferon-alpha and chemotherapy, are indicated for extensive or symptomatic visceral disease (when organs are involved).
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