Kaposi Sarcoma

Kaposi sarcoma (KS) is a type of soft tissue sarcoma. It causes lesions that can form on your skin or mucus membranes that line the inside of your mouth, nose and anus. These tumors may also develop in the lining of internal organs like your liver, belly and lungs.

You can get KS if you carry human herpesvirus 8 (HHV-8) and have a weakened immune system. KS is rare. Fewer than 5,000 people living in the United States have it.

Types of Kaposi sarcoma

There are four types of Kaposi sarcoma. From most to least common, they are:

• Epidemic KS. Related to AIDS/HIV, this is the most common type of Kaposi sarcoma in the U.S.
• Classic KS. This type of KS mostly affects males over 60 who are of Mediterranean, Southwest Asian, Eastern European or Ashkenazi Jewish descent. Lesions might also spread to internal organs.
• Endemic KS. This type of KS affects people living near the African equator. It is similar to classic KS, but the age of diagnosis is usually much younger (under age 40).
• Acquired KS. You can get this type if you carry HHV-8 and take immunosuppressants after an organ or bone marrow transplant.

Symptoms of Kaposi sarcoma

Kaposi sarcoma symptoms can vary depending on the location of the lesions. You might develop:

• Abnormal spots on your skin that appear in one or more areas (these patchy spots might be smooth or bumpy)
• Belly pain or blood in your poop from lesions in your belly
• Constipation, diarrhea or vomiting from lesions in your digestive system
• Coughing up blood or difficulty breathing because there are lesions in your lungs
• Lymphedema, which can happen when lesions block one of your lymph nodes
• Mouth pain when eating due to lesions in your mouth

Kaposi sarcoma causes

Kaposi sarcoma happens when HHV-8 infects the cells that line your blood and lymphatic vessels. HHV-8 is a rare disease that turns healthy cells into cancerous cells. Certain factors increase the risk of HHV-8 becoming Kaposi sarcoma.

Risk factors

Risk factors for Kaposi sarcoma include:

• Age. Kaposi sarcoma mostly affects those between ages 40 and 70.
• Ethnicity. You have a higher risk for KS if you’re of Mediterranean, Southwest Asian, Eastern European or Ashkenazi Jewish descent.
• Immune deficiency. You’re more likely to develop KS if you have a weakened immune system. This can happen from taking immunosuppressants or having conditions like HIV/AIDS.
• Location. You have a higher risk for endemic KS if you live near the equator in Africa.
• Sex. Males are more likely to develop Kaposi sarcoma.
• Sexual activity. Having unprotected sex can increase your risk of contracting HHV-8 and HIV. These viruses typically spread through bodily fluids.

Complications of Kaposi sarcoma

Kaposi sarcoma can lead to complications, which may include:

• Anemia
• Breathing issues
• Pain
• Second cancer
• Skin abnormalities
• Skin swelling and disfigurement

How doctors diagnose Kaposi sarcoma

A healthcare provider will start with a physical examination. They’ll check your skin for lesions and ask about your symptoms and health history.

Because Kaposi sarcoma is so rare, it can take a while to get an accurate diagnosis. You may need to see several different types of healthcare providers and specialists. Ask your care team what to expect in your situation.

Tests that are used

To get an accurate diagnosis, your healthcare provider will need to run tests, which may include:

• Biopsy. Your provider takes a small tissue sample and sends it to a pathologist for testing.
• Bronchoscopy. This test helps your provider look for KS in your windpipe and the airways that lead to your lungs.
• Chest X-ray. They’ll take images of your lungs to look for KS lesions. You may also need a CT scan.
• Endoscopy. This test helps your healthcare provider look inside your stomach or intestines for KS lesions. Depending on your situation, you might need an upper endoscopy, a colonoscopy or both.
• Immunohistochemistry. This lab test looks for a specific protein called LANA-1. It’s associated with HHV-8. It helps your provider tell the difference between KS and similar lesions.

How is Kaposi sarcoma treated?

It depends on your situation. Your healthcare team will create a personalized plan unique to you. Treatments may include:

• Antiretroviral therapy
• Chemotherapy
• Cryotherapy
• Radiation therapy
• Surgery
• Targeted therapy

The treatment that’s right for you depends on a few different factors, like the type of KS you have, the number and location of lesions, and your overall health. Your oncologist can tell you what to plan for in your case.

When should I see my healthcare provider?

Kaposi sarcoma can come back (recur) after treatment. So, you’ll have regular follow-ups so your healthcare provider can monitor your health and check for signs of new cancer.

If you’re living with HIV/AIDS or have had an organ transplant, your regular check-ups will include screenings for recurring KS.

Can Kaposi sarcoma be prevented?

According to the American Cancer Society, taking steps to reduce your HIV risk may help prevent Kaposi sarcoma.

If you have HIV, highly active antiretroviral therapy can reduce your risk of developing Kaposi sarcoma and AIDS.

What can I expect if I have Kaposi sarcoma?

Your outlook depends on many factors. But like most cancers, early detection and treatment give you the best chance for long-term survival.

The overall five-year survival rate for Kaposi sarcoma is 75%. That means that 75% of people who have KS are still alive five years after their diagnosis.

Keep in mind that cancer survival rates are only estimates. They can’t predict how long you’ll live or how well you’ll respond to treatment. To learn more about survival rates and what they mean for you, talk to your healthcare provider.

How can I take care of myself?

If you have Kaposi sarcoma, maintaining a healthy immune system is the best thing you can do to take care of yourself. You can support your immune system by:

• Eating plenty of nutritious foods, like fresh fruits and veggies
• Getting enough sleep
• Limiting beverages that contain alcohol
• Managing stress
• Quitting smoking
• Staying up to date on vaccines
• Washing your hands often

I’m an organ transplant recipient. How can I reduce my risk of Kaposi sarcoma?

Talk to your healthcare provider if you have HHV-8. There are immunosuppressant medications that protect your transplanted organ without increasing your risk of Kaposi sarcoma.

Is Kaposi sarcoma an autoimmune disease?

Kaposi sarcoma isn’t an autoimmune disease. It’s a type of cancer that usually affects people with weakened immune systems.

In other words, it happens when your immune system is already weak, not when your body attacks its own tissues.