Kaposi sarcoma

Kaposi sarcoma is a type of cancer that forms in the lining of blood vessels and lymph vessels. The cancer forms growths of cells, called lesions, on the skin. The lesions often form on the face, arms and legs. The lesions may look pink, red, purple or brown.

Lesions also can appear on the genitals or in the mouth. In severe Kaposi sarcoma, lesions can be in the digestive tract and lungs.

The cause of Kaposi sarcoma is infection with the virus human herpes virus 8, also called HHV-8. In healthy people, this infection usually causes no symptoms because the immune system keeps it under control. In someone with a weakened immune system, however, HHV-8 can lead to Kaposi sarcoma.

Types of Kaposi sarcoma include:

• AIDS-related or epidemic Kaposi sarcoma. This type happens in people infected with human immunodeficiency virus, also called HIV. HIV is the virus that causes AIDS.
• Transplant-associated or iatrogenic Kaposi sarcoma. This type happens in people who take medicine to control the immune system after an organ transplant.
• Classic Kaposi sarcoma. This type occurs in older adults of Eastern European, Mediterranean and Middle Eastern descent. It usually grows slowly and can cause swelling in areas such as the legs.
• Endemic Kaposi sarcoma. This type affects young people in Africa. It can grow slowly on the skin or quickly inside the body.

Kaposi sarcoma signs and symptoms include:

• A growth on the skin that may be raised or flat.
• A growth on the skin that looks red, purple or brown in color.

The growths, called lesions, most often happen on the face, arms or legs. They usually don't cause discomfort.

If Kaposi sarcoma isn't treated, the lesions can get bigger. They may cause:

• Swelling in the lower legs caused by blood flow problems.
• Enlarged lymph nodes.
• Skin that appears red or purple in color and may be painful and itchy.

Kaposi sarcoma also can affect areas you can't see. It can grow in the digestive tract or lungs. When Kaposi sarcoma happens in the digestive tract, symptoms may include:

• Diarrhea.
• Nausea.
• Stomach pain.
• Vomiting.
• Weight loss.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have symptoms that worry you.

The human herpes virus 8 causes Kaposi sarcoma. Healthcare professionals believe this virus, also called HHV-8, spreads from person to person through saliva. It also may be passed through blood.

When a healthy person gets the HHV-8 virus, that person's immune system is likely to control it. The virus may stay in the body, but it doesn't cause any problems. If something happens to weaken the immune system, the virus may no longer be controlled. This can lead to Kaposi sarcoma.

Risk factors for Kaposi sarcoma include:

• HIV infection. HIV is the virus that causes AIDS.
• Older age. Kaposi sarcoma can happen at any age. It's most common in adults between 50 and 70.
• Living in certain parts of the world. Kaposi sarcoma is rare in the United States. It's most common in the Mediterranean, Eastern Europe and sub-Saharan Africa.
• Medicines that weaken the immune system. Some conditions are treated with medicine to control the immune system. Medicine that works in this way often is used after organ transplant surgery.

Taking a piece of skin for testing

A healthcare professional may recommend removing a small piece of a skin lesion for testing. This procedure is called a skin biopsy. The sample is sent to a lab for testing. Lab tests can look for signs of cancer.

A skin biopsy can confirm Kaposi sarcoma.

Testing for Kaposi sarcoma inside the body

Other tests might be needed to look for Kaposi sarcoma in the lungs or the digestive tract.

Tests to find Kaposi sarcoma in the digestive tract might include:

• Fecal occult blood test. This test detects hidden blood in stool. If it shows hidden blood, other tests might be needed to find the source. Other tests include an endoscopy or colonoscopy. These tests are used to see if Kaposi sarcoma is causing the bleeding.
• Endoscopy. In this test, a thin tube, called an endoscope, is passed through the mouth. It allows a healthcare professional to look at the esophagus, stomach and first part of the small intestine.
• Colonoscopy. In this test, a thin tube called a colonoscope goes through the rectum and into the colon. It allows a health professional to look at the walls of these organs.
• CT scan. This imaging test uses X-rays to make detailed images of the inside of the body. A CT of the abdomen and pelvis can show the digestive tract.

Tests to find Kaposi sarcoma in the lungs might include:

• Chest X-ray. A chest X-ray might show something unusual in the lungs. If so, a CT scan of the chest or a bronchoscopy might be used to see if the unusual finding is Kaposi sarcoma.
• CT scan. This imaging test uses X-rays to make detailed images of the inside of the body. A CT scan of the chest can show the lungs.
• Bronchoscopy. In this test, a thin tube called a bronchoscope passes through the nose or mouth into the lungs. This allows for viewing the lining of the airway and taking samples of lung tissue.

There's no cure for Kaposi sarcoma. But there are many treatment options that can help control it. Some people may not need treatment right away. Instead, the condition might be monitored to make sure it's not getting worse. Treatment depends on:

• The type of Kaposi sarcoma.
• The number of lesions and where they are.
• The effects of the lesions, such as causing pain or getting in the way of eating or breathing.
• Your overall health.

Treatment for AIDS-related Kaposi sarcoma

Thanks to better antiviral medicines to treat AIDS and ways to prevent it, Kaposi sarcoma has become less common and less severe in people with AIDS. Taking antiviral medicines can lower the amount of the virus that causes HIV/AIDS and make the immune system stronger. This might be the only treatment needed for Kaposi sarcoma.

Treatment for transplant-related Kaposi sarcoma

Some people with transplant-related Kaposi sarcoma may be able to stop taking the medicines that are controlling the immune system or switch to another medicine.

Treatment for small skin lesions

Treatments for small skin lesions might include:

• Minor surgery, also called excision.
• Freezing treatment, called cryotherapy.
• Radiation therapy.
• An injection of the chemotherapy medicine vinblastine into lesions.
• Applying a medicine cream or gel to the skin.

Lesions treated in any of these ways are likely to return within a couple of years. When this happens, treatment often can be repeated.

Treatment if there are many skin lesions

If Kaposi sarcoma causes many skin lesions, other treatments might be needed, such as:

• Radiation therapy. Radiation therapy uses powerful energy beams to kill cancer cells. This is a treatment option if there are many skin lesions, but not enough to need chemotherapy.
• Chemotherapy. Chemotherapy uses strong medicines to kill cancer cells. Chemotherapy might be an option when Kaposi sarcoma affects multiple parts of the body. For Kaposi sarcoma that's getting worse quickly, chemotherapy might help.

Start by seeing a doctor or other healthcare professional if you have any symptoms that worry you. If your health professional thinks you might have Kaposi sarcoma, you might need to see a specialist. Specialists who care for people with Kaposi sarcoma include:

• Doctors who treat conditions caused by infections, called infectious disease specialists.
• Doctors who treat skin conditions, called dermatologists.
• Doctors who treat cancer, called oncologists.

What you can do

When you make the appointment, ask if there's anything you need to do in advance.

Make a list of:

• Your symptoms, including when you noticed the skin growth and how it may have changed over time.
• Key personal information, including your medical history, recent life changes and family medical history.
• All medicines, vitamins or other supplements you take, including the doses.
• Questions to ask your healthcare professional.

You may want to bring a friend or family member along to help you remember the information you're given.

For Kaposi sarcoma, some basic questions to ask include:

• What's likely causing my symptoms?
• Other than the most likely cause, what are other possible causes for my symptoms?
• What tests do I need?
• Does my condition have a cure?
• What's the best course of action?
• I have these other health conditions. How can I best manage them together?
• Should I see a specialist?
• Are there brochures or other printed material I can have? What websites do you recommend?
• What would happen if I choose not to have treatment?

Don't hesitate to ask other questions.

What to expect from your doctor

Your healthcare professional may ask questions about your symptoms, such as:

• When did your symptoms begin?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?