Kaposi Sarcoma – Causes, Symptoms, Treatment

Kaposi sarcoma is a cancer that causes lesions (abnormal tissue) 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 with HHV-8 do not get Kaposi sarcoma. People with HHV-8 are more likely to develop Kaposi sarcoma if their immune system is weakened by disease, such as human immunodeficiency virus (HIV), or by drugs given after an organ transplant.

There are several types of Kaposi sarcoma. The two types discussed in this summary include:

• Classic Kaposi sarcoma.
• Epidemic Kaposi sarcoma (HIV-associated Kaposi sarcoma).

Tests that examine the skin, lungs, and gastrointestinal tract are used to diagnose Kaposi sarcoma.

The following tests and procedures may be used:

• Physical exam and health 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.
• 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 sarcoma in the lungs.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
  One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin:

  • Excisional biopsy: A scalpel is used to remove the entire skin growth.
  • Incisional biopsy: A scalpel is used to remove part of a skin growth.
  • Core biopsy: A wide needle is used to remove part of a skin growth.
  • Fine-needle aspiration (FNA) biopsy: A thin needle is used to remove part of a skin growth.
  An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs.

  • Endoscopy for biopsy: 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 sarcoma lesions in the gastrointestinal tract.
  • Bronchoscopy for biopsy: 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 disease. This is used to find Kaposi sarcoma lesions in the lungs.

After Kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The following tests and procedures may be used to find out if cancer has spread to other parts of the body:

• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, liver, and spleen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• PET scan (positron emission tomography scan): A procedure to find malignant lesions in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant lesions show up brighter in the picture because they are more active and take up more glucose than normal cells do. This imaging test checks for signs of cancer in the lung, liver, and spleen.
• CD34 lymphocyte count: A procedure in which a blood sample is checked to measure the amount of CD34 cells (a type of white blood cell). A lower than normal amount of CD34 cells can be a sign the immune system is not working well.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

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

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

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

Signs of classic Kaposi 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 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.

Patients with human immunodeficiency virus (HIV) are at risk of developing epidemic Kaposi sarcoma (HIV-associated Kaposi sarcoma).

Acquired immunodeficiency syndrome (AIDS) is caused by HIV, which attacks and weakens the body’s immune system. A weakened immune system is unable to fight infection and disease. People with HIV have an increased risk of infection and cancer.

A person with HIV and certain types of infection or cancer, such as Kaposi sarcoma, is diagnosed as having AIDS. Sometimes, a person is diagnosed with AIDS and epidemic Kaposi sarcoma at the same time.

The use of drug therapy called highly active antiretroviral therapy (HAART) reduces the risk of epidemic Kaposi sarcoma in patients with HIV.

HAART is a combination of several drugs used to lessen the damage to the immune system caused by HIV infection. Treatment with HAART reduces the risk of epidemic Kaposi sarcoma, although it is possible to for a person to develop epidemic Kaposi sarcoma while taking HAART.

For information about AIDS and its treatment, see the AIDSinfo website.

Signs of epidemic Kaposi sarcoma can include lesions that form in many parts of the body.

The signs of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following:

• Skin.
• Lining of the mouth.
• Lymph nodes.
• Stomach and intestines.
• Lungs and lining of the chest.
• Liver.
• Spleen.

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.

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

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.

Six types of standard treatment are used to treat Kaposi sarcoma:

Treatment of epidemic Kaposi sarcoma combines treatment for Kaposi sarcoma with treatment for acquired immunodeficiency syndrome (AIDS). The six types of standard treatment used to treat Kaposi sarcoma include:

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Treatment for Kaposi sarcoma may cause side effects.

Patients may want to think about taking part in a clinical trial.

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.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

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.

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). Treatment of Classic Kaposi Sarcoma