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 Overview of Herpesvirus Infections Some common viral infections are caused by herpesviruses. Eight different herpesviruses infect people: Three herpesviruses— herpes simplex virus type 1, herpes simplex virus type 2, and varicella-zoster... read more . 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 Symptoms Human immunodeficiency virus (HIV) infection is a viral infection that progressively destroys certain white blood cells and is treated with antiretroviral medications. If untreated, it can cause... read more
(which accounts for most of the cases in the United States).
Symptoms of Kaposi Sarcoma
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
Men over age 60 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.
In the other groups of people, 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.
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 of Kaposi Sarcoma
Punch biopsy
Doctors usually recognize Kaposi sarcoma by its appearance. A punch biopsy Biopsy Doctors can identify many skin disorders simply by looking at the skin. A full skin examination includes examination of the scalp, nails, and mucous membranes. Sometimes the doctor uses a hand-held... read more 
, 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.
Treatment of Kaposi Sarcoma
For people with one or two spots, various removal methods
For people with many spots, many affected areas, or affected lymph nodes, radiation therapy and chemotherapy
For the AIDS-associated type, antiretroviral drugs and removal methods or antiretroviral drugs and chemotherapy drugs
Treatment varies depending on the type of sarcoma.
Classic Kaposi sarcoma
Older men with slow-growing classic 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 into the spots 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 Radiation Therapy for Cancer Radiation is a form of intense energy generated by a radioactive substance, such as cobalt, or by specialized equipment, such as an atomic particle (linear) accelerator. Radiation preferentially... read more and chemotherapy Chemotherapy and Other Systemic Cancer Treatments Systemic treatments are those that have effects throughout the body rather than being applied directly to the cancer. Chemotherapy is a form of systemic treatment that uses drugs to kill cancer... read more . Kaposi sarcoma commonly returns and is difficult to completely cure.
People who have a more aggressive form that affects internal organs, but whose immune system is normal, often respond to interferon alfa or, more often, chemotherapy drugs Chemotherapy and Other Systemic Cancer Treatments Systemic treatments are those that have effects throughout the body rather than being applied directly to the cancer. Chemotherapy is a form of systemic treatment that uses drugs to kill cancer... read more given by vein.
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 Antiretroviral Treatment of Human Immunodeficiency Virus (HIV) Infection Antiretroviral medications used to treat human immunodeficiency virus (HIV) infection aim to do the following: Reduce the amount of HIV RNA (viral load) in the blood to an undetectable amount... read more (ART) helps. Some people who have the AIDS-associated type need only ART and the removal methods noted above. Other people who have the AIDS-associated type may need chemotherapy drugs (such as doxorubicin or paclitaxel) by vein in addition to ART. 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.
More Information
The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
American Cancer Society: Kaposi Sarcoma: Information about Kaposi sarcoma, including detection, prevention, treatment options, and other resources
Drugs Mentioned In This Article
| Generic Name | Select Brand Names |
|---|---|
imiquimod |
Aldara, Zyclara |
vinblastine |
Velban |
sirolimus |
HYFTOR, Rapamune |
doxorubicin |
Adriamycin, Adriamycin PFS, Adriamycin RDF, Rubex |
paclitaxel |
Onxol , Taxol |
