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Kaposi sarcoma is a cancer that causes multiple flat pink, red, or purple patches or bumps on the skin. It is caused by herpesvirus type 8.
One or a few spots may appear on the toes or a leg, or spots may appear anywhere on the body or in the mouth or genital areas, then spread to other areas, including internal organs.
Although this cancer can often be identified by sight, doctors usually also do a biopsy.
Spots may be removed or treated with radiation therapy, but if the cancer is aggressive, treatment includes chemotherapy drugs or interferon alfa.
There are four types of Kaposi sarcoma. The types occur in several distinct groups of people and act differently in each group. It occurs in the following:
Classic: Affects older men, usually of Italian, Eastern European, or Jewish heritage
Endemic (occurs regularly in a certain place): Affects children and young adults from certain parts of Africa
Immunosuppressive: Affects people whose immune system is weakened due to immunosuppressants given after organ transplantation
AIDS-associated: Affects people with AIDS (which accounts for most of the cases in the United States)
Kaposi sarcoma usually appears as a single purple, pink, or red spot on the toes or a leg. Swelling may be present. The cancer may grow to several inches or more as a blue-violet to black, flat or slightly raised area that tends to bleed and break open. Older (over age 60) men with the classic type may develop several additional spots on the leg, but the cancer rarely spreads to other parts of the body and is almost never fatal.
In the other groups, Kaposi sarcoma is more aggressive. Similar-appearing spots develop, but they are often multiple and may occur anywhere on the body.
Within several months, people with the AIDS-associated type develop spots that spread to other parts of the body, such as the face and torso, often including the mouth, where they cause pain with eating. They may also develop in lymph nodes and internal organs, especially the digestive tract, where they can cause diarrhea and internal bleeding that leads to blood in the stool.
The immunosuppressive type typically develops several years after organ transplantation and is severe.
The endemic type is usually sudden, severe, and fatal in children. In adults, the endemic type tends to cause symptoms similar to those of the classic type.
Doctors usually recognize Kaposi sarcoma by its appearance. A biopsy is usually done to confirm the diagnosis.
Older men with slow-growing Kaposi sarcoma in one or two spots may have the tumors removed surgically or with extreme cold (cryosurgery). People with multiple spots or affected lymph nodes receive radiation therapy. Some people with very few spots and no other symptoms may choose to receive no treatment unless the condition spreads.
People who have a more aggressive form, but whose immune system is normal, often respond to interferon alfa or chemotherapy drugs.
In people taking immunosuppressants, the tumors sometimes disappear when immunosuppressants are stopped. However, if these drugs must be continued because of the person’s underlying condition, surgery, chemotherapy, and radiation therapy are used. People may also be given sirolimus. These treatment methods are less successful than in people with a healthy immune system.
In people with AIDS, treatment with chemotherapy and radiation has not been very successful. However, intensive treatment with AIDS drugs (combinations of drugs referred to as highly active antiretroviral therapy [HAART]) helps, provided that people’s immune system improves because of the treatment. People with the AIDS-associated type may also be given interferon alfa, doxorubicin, or paclitaxel by vein. In general, treating Kaposi sarcoma does not prolong the lives of most people with AIDS.
The endemic type is difficult to treat. Doctors typically make sure people are comfortable and free of pain and that their symptoms are fully treated.
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