Source: National Cancer Institute Research, www.cancer.gov
Kaposi sarcoma is a cancer that causes lesions (abnormaltissue) to grow in the skin; the mucous membranes lining the mouth, nose, and throat; lymph nodes; or other organs. The lesions are usually purple and are made of cancer cells, new blood vessels, red blood cells, and white blood cells. Kaposi sarcoma is different from other cancers in that lesions may begin in more than one place in the body at the same time.
Human herpesvirus-8 (HHV-8) is found in the lesions of all patients with Kaposi sarcoma. This virus is also called Kaposi sarcoma herpesvirus (KSHV). Most people infected with HHV-8 do not get Kaposi sarcoma. Those infected with HHV-8 who are most likely to develop Kaposi sarcoma have immune systems weakened by disease or by drugs given after an organ transplant.
There are several types of Kaposi sarcoma, including:
The following tests and procedures may be used:
One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin:
An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs.
The following tests and procedures may be used to find out if cancer has spread to other parts of the body:
The prognosis (chance of recovery) and treatment options depend on the following:
Classic Kaposi sarcoma is a rare disease that gets worse slowly over many years.
Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions may form in other parts of the body, such as the stomach, intestines, or lymph nodes. The lesions usually don't cause any symptoms, but may grow in size and number over a period of 10 years or more. Pressure from the lesions may block the flow of lymph and blood in the legs and cause painful swelling. Lesions in the digestive tract may cause gastrointestinal bleeding.
Some patients with classic Kaposi sarcoma may develop another type of cancer before the Kaposi sarcoma lesions appear or later in life. Most often, this second cancer is non-Hodgkin lymphoma. Frequent follow-up is needed to watch for these second cancers.
African Kaposi sarcoma is a fairly common form of the disease found in young adult males who live near the equator in Africa. Signs of African Kaposi sarcoma can be the same as classic Kaposi sarcoma. However, African Kaposi sarcoma can also be found in a much more aggressive form that may cause sores on the skin and spread from the skin to the tissues to the bone. Another form of Kaposi sarcoma that is common in young children in Africa does not affect the skin but spreads through the lymph nodes to vitalorgans, and quickly becomes fatal.
This type of Kaposi sarcoma is not common in the United States and treatment information is not included in this summary.
Immunosuppressive therapy–related Kaposi sarcoma is found in patients who have had an organtransplant (for example, a kidney, heart, or liver transplant). These patients take drugs to keep their immune systems from attacking the new organ. When the body's immune system is weakened by these drugs, diseases like Kaposi sarcoma can develop.
Immunosuppressive therapy–related Kaposi sarcoma often affects only the skin, but may also occur in the mucous membranes or certain other organs of the body.
This type of Kaposi sarcoma is also called transplant-related or acquired Kaposi sarcoma.
Epidemic Kaposi sarcoma occurs in patients who have acquired immunodeficiency syndrome (AIDS). AIDS is caused by the human immunodeficiency virus (HIV), which attacks and weakens the immune system. When the body's immune system is weakened by HIV, infections and cancers such as Kaposi sarcoma can develop.
Most cases of epidemic Kaposi sarcoma in the United States have been diagnosed in homosexual or bisexual men infected with HIV.
The signs of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following:
Kaposi sarcoma is sometimes found in the lining of the mouth during a regular dental check-up.
In most patients with epidemic Kaposi sarcoma, the disease will spread to other parts of the body over time. Fever, weight loss, or diarrhea can occur. In the later stages of epidemic Kaposi sarcoma, life-threatening infections are common.
HAART (highly active antiretroviral therapy) is a combination of several drugs that block HIV and slow down the development of AIDS and AIDS-related Kaposi sarcoma. For information about AIDS and its treatment, see the AIDSinfo Web site.
There is a type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of HIVinfection. This type of Kaposi sarcoma progresses slowly, with new lesions appearing every few years. The lesions are most common on the arms, legs, and genitals, but can develop anywhere on the skin.
This type of Kaposi sarcoma is rare and treatment information is not included in this summary.
RecurrentKaposi sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the skin or in other parts of the body.
Different types of treatments are available for patients with Kaposi sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
For the treatment of epidemic Kaposi sarcoma, highly active antiretroviral therapy (HAART) is used to slow the progression of AIDS. HAART may be combined with anticancer drugs and medicines that prevent and treat infections.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated.
Certain types of external radiation therapy are used to treat Kaposi sarcoma lesions. Photon radiation therapy treats lesions with high-energy light. Electron beam radiation therapy uses tiny negatively charged particles called electrons.
The following surgical procedures may be used for Kaposi sarcoma to treat small, surface lesions:
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). To treat local Kaposi sarcoma lesions, such as in the mouth, anticancer drugs may be injected directly into the lesion (intralesional chemotherapy). Sometimes the chemotherapy is given as a topical agent (applied to the skin as a gel.) The way the chemotherapy is given depends on the type of cancer being treated.
Liposomal chemotherapy uses liposomes (very tiny fat particles) to carry anticancer drugs. Liposomal doxorubicin is used to treat Kaposi sarcoma. The liposomes build up in Kaposi sarcoma tissue more than in healthy tissue, and the doxorubicin is released slowly. This increases the effect of the doxorubicin and causes less damage to healthy tissue.
See Drugs Approved for Kaposi Sarcoma for more information.
Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent used to treat Kaposi sarcoma.
See Drugs Approved for Kaposi Sarcoma for more information.
Information about clinical trials is available from the NCI Web site.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodytherapy is one type of targeted therapy being studied in the treatment of Kaposi sarcoma.
Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. These may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
Bevacizumab is a monoclonal antibody that is being studied in the treatment of Kaposi sarcoma.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment for single lesions may include the following:
Treatment for lesions all over the body may include the following:
Treatment for Kaposi sarcoma that affects lymph nodes or the gastrointestinal tract usually includes chemotherapy with or without radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with classic Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
Treatment for immunosuppressive therapy–related Kaposi sarcoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with immunosuppressive treatment related Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
Treatment for epidemic Kaposi sarcoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with AIDS-related Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
Treatment for recurrentKaposi sarcoma depends on which type of Kaposi sarcoma the patient has. Treatment may include a clinical trial of a new therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about Kaposi sarcoma, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following: