(Kaposi's Sarcoma; Multiple Idiopathic Hemorrhagic Sarcoma)
Topic Resources
Kaposi sarcoma is a skin cancer
that causes multiple flat pink, red, or purple patches or bumps on the
skin. It is caused by human herpesvirus type 8 infection.
- 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 many types of human herpesviruses. One of them, type 8, can cause Kaposi sarcoma, particularly in people with a weakened immune system.
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 Kaposi sarcoma: This type affects older men, usually of Italian, Eastern European, or Jewish heritage.
- Endemic Kaposi sarcoma: Endemic means this cancer occurs regularly in a certain place. It affects children and young adults from certain parts of Africa.
- Immunosuppressive Kaposi sarcoma: This type affects people whose immune system is weakened by immunosuppressants given after organ transplantation.
- AIDS-associated Kaposi sarcoma (epidemic Kaposi sarcoma): This type affects people with AIDS (which accounts for most of the cases in the United States).
Symptoms
Kaposi sarcoma usually appears as purple, pink, or red spots or
bumps on the skin. The cancer may grow to several inches or more as a
blue-violet to black, flat or slightly raised area. Swelling may be
present. Sometimes the cancer grows deeper into soft tissues and invades
bone. Cancer of mucosal surfaces, such as in the mouth, are blue to
violet in color. In the digestive tract, the cancer can sometimes bleed
excessively but usually causes no symptoms.
Classic Kaposi sarcoma
Older (over age 60) men with the classic type may develop several
additional spots on the legs, but the cancer rarely spreads to other
parts of the body and is almost never fatal.
Classic Kaposi Sarcoma
Classic
Kaposi sarcoma is a tumor that occurs most often in older men,
typically on the skin of the lower legs. It is slow-growing and causes a
small number of purple, pink, or red spots that sometimes combine into
blue-violet to black patches as shown here.
Image provided by Thomas Habif, MD.
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.
AIDS-associated Kaposi sarcoma
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.
The spots may also develop in lymph nodes and internal organs,
especially the digestive tract, where they can cause internal bleeding
that leads to blood in the stool. Sometimes Kaposi sarcoma is the first
symptom of AIDS.
AIDS-Associated Kaposi Sarcoma
AIDS-associated
Kaposi sarcoma is an aggressive cancer that occurs in people with HIV
infection. It can develop on the skin, mouth (as shown here on the upper
gums), digestive tract and lymph nodes.
The tumors cause blue or purple spots, patches, or lumps.
The tumors cause blue or purple spots, patches, or lumps.
Image courtesy of Sol Silverman, Jr., via the Public Health Image Library of the Centers for Disease Control and Prevention.
Immunosuppressive Kaposi sarcoma
The immunosuppressive type typically develops several years after organ transplantation and is severe.
Endemic Kaposi sarcoma
In children, the endemic type usually involves the lymph nodes.
The children may or may not have spots on the skin. The disease is
usually sudden, severe, and fatal.
In adults, the endemic type tends to cause slow-growing spots and
patches on the skin similar to those of the classic type. The cancer
rarely spreads to other parts of the body and is not likely to be fatal.
Diagnosis
-
Punch biopsy
Doctors usually recognize Kaposi sarcoma by its appearance. A punch biopsy,
in which a small piece of skin is removed for examination under a
microscope, is usually done to confirm the diagnosis of Kaposi sarcoma.
For people with AIDS or immunosuppression, doctors do other tests
to determine where the cancer has spread. Doctors usually do a computed
tomography (CT) scan of the chest and the abdomen and do other tests
depending on the person's symptoms.
Punch Biopsy
Treatment
-
For people with one or two spots, removal
-
For people with many spots, many affected areas, or affected lymph nodes, radiation therapy and chemotherapy
-
For the AIDS-associated type, antiretroviral drugs, interferon alfa, or chemotherapy drugs
Older men with slow-growing Kaposi sarcoma in one or two spots
may have the tumors removed surgically, with extreme cold (cryosurgery),
or with an electrical current (electrocoagulation).
Imiquimod cream or injections of vinblastine or interferon alfa may also be used to remove the spots. Some people with very few spots and no other symptoms may choose to receive no treatment unless the condition spreads.
Imiquimod cream or injections of vinblastine or interferon alfa may also be used to remove the spots. Some people with very few spots and no other symptoms may choose to receive no treatment unless the condition spreads.
People who have many spots, many affected areas, or affected lymph nodes receive radiation therapy and chemotherapy. Kaposi sarcoma commonly returns and is difficult to completely cure.
People who have a more aggressive form, but whose immune system is normal, often respond to interferon alfa or chemotherapy drugs.
Immunosuppressive Kaposi sarcoma
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, the
immunosuppressants are reduced. If immunosuppressants cannot be reduced,
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.
AIDS-associated Kaposi sarcoma
In people with AIDS, treatment with chemotherapy and radiation has not been very successful. However, intensive treatment with antiretroviral therapy
(ART) helps, provided that people’s immune system improves because of
the treatment. People with the AIDS-associated type may also be given
interferon alfa or chemotherapy drugs (such as doxorubicin or paclitaxel) by vein. In general, treating Kaposi sarcoma does not prolong the lives of most people with AIDS.
Endemic Kaposi sarcoma
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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