Kaposi's Sarcoma

Summary Type: Treatment
Summary Audience: Patients
Summary Language: English
Summary Description: Expert-reviewed information summary about the treatment of Kaposi's sarcoma.


Kaposi's Sarcoma

General Information About Kaposi's Sarcoma

Kaposi's sarcoma is a disease in which malignant (cancer) cells form in the tissue lining the lymph vessels under the skin or in mucous membranes.

Kaposi's sarcoma is a cancer that causes lesions (abnormal tissue ) to grow under the skin, in the lining of the mouth, nose, and throat , or in other organs . The lesions are usually purple and are made of cancer cells , new blood vessels , and white blood cells . Kaposi's 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's sarcoma. This virus is also called Kaposi's sarcoma herpesvirus (KSHV). Most people infected with HHV-8 do not get Kaposi's sarcoma. Those infected with HHV-8 who are most likely to develop Kaposi's sarcoma have immune systems weakened by disease or by drugs given after an organ transplant .

There are several types of Kaposi's sarcoma, including:

  • Classic Kaposi's sarcoma.
  • African Kaposi's sarcoma.
  • Immunosuppressive treatment-related Kaposi's sarcoma.
  • Epidemic Kaposi's sarcoma.
  • Nonepidemic Kaposi's sarcoma.
Tests that examine the skin, lungs, and gastrointestinal tract are used to detect (find) and diagnose Kaposi's sarcoma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking skin and lymph nodes for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
  • Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
  • Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. This is used to find Kaposi's sarcoma in the lungs .
  • Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. This is used to find Kaposi's sarcoma lesions in the gastrointestinal tract.
  • Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The type of Kaposi's sarcoma.
  • The general health of the patient, especially the immune system.
  • Whether the cancer has spread.
  • Whether the cancer has just been diagnosed or has recurred (come back).

Classic Kaposi's Sarcoma

Classic Kaposi's sarcoma is found most often in older men of Italian or Eastern European Jewish origin.

Classic Kaposi's sarcoma is a rare disease that gets worse slowly over many years.

Symptoms of classic Kaposi's sarcoma may include slow-growing lesions on the legs and feet.

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.

Another cancer may develop.

Some patients with classic Kaposi's sarcoma may develop another type of cancer before the Kaposi's sarcoma lesions appear or later in life. Most often, this second cancer is non-Hodgkin's lymphoma . Frequent follow-up is needed to watch for these second cancers.

African Kaposi's Sarcoma

African Kaposi's sarcoma is a fairly common form of the disease found in young adult males who live near the equator in Africa. Symptoms of African Kaposi's sarcoma can be the same as classic Kaposi's sarcoma. However, African Kaposi's 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's sarcoma that is common in young children in Africa does not affect the skin but spreads through the lymph nodes to vital organs , and quickly becomes fatal.

This type of Kaposi's sarcoma is not common in the United States and treatment information is not included in this summary.

Immunosuppressive Treatment-related Kaposi's Sarcoma

Immunosuppressive treatment-related Kaposi's sarcoma is found in patients who have had an organ transplant (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's sarcoma can develop.

Immunosuppressive treatment-related Kaposi's sarcoma often affects only the skin, but may also occur in the mucous membranes or other organs.

This type of Kaposi's sarcoma is also called transplant-related or acquired Kaposi's sarcoma.

Epidemic Kaposi's Sarcoma

Epidemic Kaposi's sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS).

Epidemic Kaposi's 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 like Kaposi's sarcoma can develop.

Most cases of epidemic Kaposi's sarcoma in the United States have been diagnosed in homosexual or bisexual men with HIV infection.

Symptoms of epidemic Kaposi's sarcoma include lesions that may spread to many parts of the body.

Symptoms of epidemic Kaposi's sarcoma include lesions on different parts of the body, including any of the following:

Kaposi's sarcoma is sometimes found in the lining of the mouth during a regular dental check-up.

In most patients with epidemic Kaposi's 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's sarcoma, life-threatening infections are common.

The use of drug therapy called HAART reduces the risk of epidemic Kaposi's sarcoma in HIV-infected patients.

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's sarcoma. For information about AIDS and its treatment, see the AIDSinfo Web site.

Nonepidemic Gay-related Kaposi's Sarcoma

There is a type of nonepidemic Kaposi's sarcoma that develops in homosexual men who have no signs or symptoms of HIV infection . This type of Kaposi's 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's sarcoma is rare and treatment information is not included in this summary.

Recurrent Kaposi's Sarcoma

Recurrent Kaposi's 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.

Treatment Option Overview

There are different types of treatment for patients with Kaposi's sarcoma.

Different types of treatments are available for patients with Kaposi's sarcoma . Some treatments are standard (the currently used treatment), and some are being tested in clinical trials . Before starting treatment, patients may want to think about taking part in a clinical trial. 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.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Treatment of epidemic Kaposi's sarcoma combines treatment for Kaposi's sarcoma with treatment for AIDS.

For the treatment of epidemic Kaposi's 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 .

Four types of standard treatment are used to treat Kaposi's sarcoma:
Radiation therapy

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's sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. Electron beam radiation therapy uses tiny negatively charged particles called electrons. Photon radiation therapy and electron beam radiation therapy will kill cancer cells near the surface of the body without harming deeper tissues and bone.

Surgery

The following surgical procedures may be used for Kaposi's sarcoma to treat small, surface lesions:

  • Local excision : The cancer is cut from the skin along with a small amount of normal tissue around it.
  • Electrodesiccation and curettage : The tumor is cut from the skin with a curette (a sharp, spoon-shaped tool). A needle-shaped electrode is then used to treat the area with an electric current that stops the bleeding and destroys cancer cells that remain around the edge of the wound . The process may be repeated one to three times during the surgery to remove all of the cancer.
  • Cryosurgery : A treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy.
Chemotherapy

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 spinal column , 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's 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 carries anticancer drugs in liposomes (very tiny fat particles). Liposomal chemotherapy builds up in Kaposi's sarcoma tissue more than in healthy tissue, and is slowly released. This increases the effect of the drug and causes less damage to healthy tissue.

Biologic therapy

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's sarcoma.

New types of treatment are being tested in clinical trials.

Information about ongoing clinical trials is available from the NCI Web site.

Treatment Options for Kaposi's Sarcoma

Classic Kaposi's Sarcoma

Treatment for single lesions may include the following:

Treatment for lesions all over the body may include the following:

Treatment for Kaposi's sarcoma that affects lymph nodes or the gastrointestinal tract usually includes chemotherapy with or without radiation therapy.

Immunosuppressive Treatment-related Kaposi's Sarcoma

Treatment for immunosuppressive treatment-related Kaposi's sarcoma may include the following:

Epidemic Kaposi's Sarcoma

Treatment for epidemic Kaposi's sarcoma may include the following:

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

Recurrent Kaposi's Sarcoma

Treatment for recurrent Kaposi's sarcoma depends on which type of Kaposi's sarcoma the patient has. Treatment may include a clinical trial of a new therapy .

Information about ongoing clinical trials is available from the NCI Web site.

Changes to This Summary (12/01/2006)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

This summary was completely reformatted and some content was added.

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PDQ also contains information on clinical trials.

Before starting treatment, patients may want to think about taking part in a clinical trial. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

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