Kaposi’s Sarcoma (KS)

What Is Kaposi’s Sarcoma?

Kaposi’s sarcoma (KS) is a form of cancer in which tumors with tiny blood vessels grow below the surface of your skin and in your mouth, nose, eyes, and anus. It can spread to your lungsliverstomachintestines, and lymph nodes, the glands that help your body fight infection.

Kaposi’s Sarcoma Symptoms

You could have a number of symptoms, depending on which parts of your body are affected by the disease.

Skin

The most visible signs of KS are lesions on your skin: flat, painless spots that are red or purple on light skin and bluish, brownish, or black on dark skin. Unlike bruises, they don't change color when you press on them. They aren't itchy, and they don't drain. They're not dangerous.

New spots may show up each week. For some people, these lesions change slowly. They may grow into raised bumps or merge together.

Mucous membranes

KS lesions can form inside your mouth and throat, causing trouble eating or swallowing. They might also happen on your eyes and under your eyelids.

Lymph nodes

When lesions block the flow of lymphatic fluid around your body, they can lead to severe swelling in your arms, legs, face, or scrotum.

Respiratory tract

Lesions inside your lungs may cause serious coughing and shortness of breath.

Digestive tract

Lesions in your stomach and intestines can lead to bleeding and blockages. You may have:

  • An upset stomach
  • Vomiting
  • Belly pain
  • Diarrhea
  • Bloody or black poop
  • Low red blood cell counts (anemia)

Types of Kaposi’s Sarcoma

There are four types:

  • Epidemic or AIDS-associated. This is the most common kind in the U.S. It affects people who have HIV. It’s known as an AIDS-defining illness because it’s on the CDC’s list of conditions that mean someone’s HIV infection has become AIDS.
  • Classic . This type affects older men of Mediterranean, Middle Eastern, or Eastern European descent.
  • Endemic. Children and young people from Africa get this kind of KS.
  • Immunosuppressive. This kind affects people who have had organ transplants and take drugs that slow down their immune system.

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Kaposi’s Sarcoma and HIV

Because people with HIV have weakened immune systems -- the body's main line of defense against germs and illnesses -- they're more likely to get certain cancers, including Kaposi’s sarcoma. Most severe cases happen when someone has AIDS, but skin lesions can show up earlier. They're a sign that your immune system isn't at full strength.

Skin lesions tend to get worse when you also have other infections.

Antiretroviral therapy for HIV also treats KS, especially early on. Anti-HIV drugs have lowered the rate of KS cases by 80% or 90% from the early 1980s.

Kaposi’s Sarcoma Causes

KS is caused by the herpes virus HHV-8, also called Kaposi’s sarcoma-related herpesvirus (KSHV). It spreads mainly through saliva, such as during sexual contact or in interactions between a mother and child.

People with healthy immune systems can carry the virus without any problems. But it triggers cancers in people with weakened immune systems.

Kaposi’s Sarcoma Risk Factors

KS affects eight times more men than women. Among people who have HIV, men who have sex with men are more likely to have the virus and to get Kaposi’s sarcoma.

Kaposi’s Sarcoma Diagnosis

Your doctor may diagnose Kaposi’s sarcoma just by looking at your skin. To confirm it, they may take a sample of tissue from a spot and look at it under a microscope, which is called a biopsy. They might also do tests including:

  • Fecal occult blood test. This looks for blood in your stool, which might mean KS is hurting your digestive tract.
  • Endoscopy /colonoscopy. If you’re having stomach trouble or belly pain, your doctor might want to look in your stomach with a lighted tube called an endoscope or in your intestines with a colonoscope.
  • Bronchoscopy. If you have trouble breathing, they might look into your airways with a thin tube called a bronchoscope.
  • Imaging tests . A CT scan or an X-ray can tell whether the cancer has spread to your lungs, your lymph nodes, or other parts of your body.

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Kaposi’s Sarcoma Treatment

Your treatment will depend on how many lesions you have, how big they are, where they are, and how well your immune system is working.

In many cases, antiretroviral therapy is the best way to treat active Kaposi’s sarcoma. It may even clear up skin lesions.

If you have just a few lesions, your doctor can cut or freeze them off. It’s not a cure for KS, but it can make your skin look better.

If you have lots of lesions or the virus is affecting many areas of your body, you might get radiation therapy. This kills the cancer cells or keeps them from growing. A machine directs radiation toward the lesions on your skin, or your doctor may put radioactive needles, seeds, or wires inside you near the cancer.

If KS has spread, you'll need medications that go through your whole body to kill the cancer. Chemotherapy drugs for Kaposi's sarcoma include:

Chemotherapy can have side effects, including hair loss, vomiting, and fatigue. It can also lower your platelet and white-blood-cell counts and raise your chances of an infection.

Another type of drug treatment, called biologic therapy, works by boosting your immune system. Your doctor may prescribe interferon alfa (Intron A) if your CD4 cell count (a type of white blood cell) is over 200 and you have a fairly healthy immune system.

Clinical trials are testing targeted therapies such as monoclonal antibody therapy and tyrosine kinase inhibitors. These treatments are aimed at attacking the cancer and keeping it from growing without hurting healthy cells.

Kaposi’s Sarcoma Outlook

Treatment can usually keep Kaposi’s sarcoma under control for many years. The lesions may shrink and fade, but they might not go away.

Overall, almost 75% of people who have KS live at least 5 years after diagnosis. If the cancer hasn’t spread, about 82% live at least 5 more years. In people whose cancer has spread to nearby areas, the 5-year survival rate is 60%. The rate is 38% if the cancer has spread farther away.

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Kaposi’s Sarcoma Prevention

There’s no vaccine to protect against HHV-8. The best way to keep from getting KS is to avoid things that put you at higher risk of HIV, such as having unprotected sex or injecting drugs with used needles. Medications called pre-exposure prophylaxis (PrEP) can also make you less likely to get HIV.

If you have HIV, antiretroviral therapy (ART) should prevent KS, especially if you start it when your CD4 count is still high. If you have advanced HIV, or AIDS, a test can tell if you also have HHV-8. ART will lower the chances that it will become KS.

If you’ve had an organ transplant, some anti-rejection drugs can also lower your chance of getting KS.

WebMD Medical Reference Reviewed by Jonathan E. Kaplan, MD on June 27, 2020

Sources

SOURCES:

National Cancer Institute: "Kaposi Sarcoma Treatment (PDQ)–Patient Version."

American Cancer Society: "Kaposi Sarcoma."

AIDS Treatment Data Network: "KS (Kaposi's Sarcoma)."

New Mexico AIDS Education & Training Center: "Kaposi's Sarcoma (KS)."

American Cancer Society: “What Is Kaposi Sarcoma?” “Can Kaposi Sarcoma Be Prevented?”

Merck Manual Consumer Version: “Kaposi Sarcoma.”

AIDSInfo: “AIDS-Defining Condition,” “CD4 T Lymphocyte.”

American Journal of Clinical Dermatology: “Diagnosis and Treatment of Kaposi Sarcoma.”

Johns Hopkins Medicine: “Kaposi Sarcoma.”

Mayo Clinic: “Kaposi’s sarcoma.”

NHS: “Kaposi’s sarcoma.”

American Society of Clinical Oncology: “Sarcoma – Kaposi.”

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