Kaposi Sarcoma

What is Kaposi sarcoma?

Kaposi sarcoma (Kaposi’s sarcoma) is a form of soft tissue sarcoma. It affects people with weakened immune systems who also carry the rare virus human herpesvirus 8 (HHV-8). The virus turns healthy cells into cancerous cells. There are different types of Kaposi sarcoma, but all types cause cancerous lesions (tumors) on people’s skin. Healthcare providers can treat this condition, but it may come back.

What are different types of Kaposi sarcoma?

There are four types of Kaposi sarcoma:

• Epidemic (AIDs-related) Kaposi sarcoma: This is the most common type of Kaposi sarcoma in the U.S. People with Kaposi sarcoma from human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome (AIDS) are likely to develop cancerous lesions throughout their bodies.
• Acquired Kaposi sarcoma: This is a rare type of Kaposi sarcoma. It affects people carrying HHV-8 who take immunosuppressants after an organ or bone marrow transplant. Acquired Kaposi sarcoma usually causes skin lesions.
• Classic Kaposi sarcoma: This sarcoma type is also known as Mediterranean sarcoma because it usually affects men and people assigned male at birth (AMAB) who are of Mediterranean, Middle Eastern and Eastern European descent. It’s more likely to affect men and people AMAB who are age 60 and older. People with this sarcoma typically have slow-growing skin lesions that grow in size and number. Lesions might also spread to internal organs.
• Endemic Kaposi sarcoma: Also known as African sarcoma, this sarcoma type affects people living in equatorial Africa. This sarcoma is like classic Kaposi sarcoma. The difference is the age of diagnosis — people generally develop endemic Kaposi sarcoma at a much younger age than those who have classic Kaposi sarcoma.

How common is this condition?

Kaposi sarcoma in the United States is rare. People develop Kaposi sarcoma because they carry HHV-8 and have weakened immune systems. Kaposi sarcoma affects 6 in 1 million people with HIV/AIDs and 1 in 200 people taking immunosuppressants after stem cell (bone marrow) transplant or organ transplant surgery.

What are Kaposi sarcoma symptoms?

Kaposi sarcoma creates patches of abnormal tissue that may appear in various places throughout your body. Lesions may develop in tissue below your skin’s surface or in mucosa that lines the inside of your mouth, nose or anus. Kaposi sarcoma may also develop in the lining of internal organs like your liver, lungs and belly.

Specific symptoms include:

• Purple, brown or red spots or patches. The patches may be bumpy or smooth. They can appear in one area of your body or in many areas.
• Lymphedema, which may happen when a lesion blocks one of your lymph nodes.
• Difficulty breathing or coughing up blood because there are lesions in your lungs.
• Blood in your poop (gastric bleeding) and belly pain from lesions in your belly.
• Diarrhea from lesions in your digestive system.
• Constipation or vomiting from lesions blocking parts of your digestive system.
• Mouth pain when eating due to lesions in your mouth.

What causes Kaposi sarcoma?

Kaposi sarcoma develops when HHV-8 infects the cells that line your blood and lymphatic vessels. Less than 10% of people in the United States carry the virus, which turns healthy cells into cancerous cells that multiply and create lesions. Certain risk factors increase the chance HHV-8 will become Kaposi sarcoma. Risk factors include:

• Ethnicity. People of Jewish or Mediterranean descent have an increased risk of classic Kaposi sarcoma. People living in equatorial Africa have an increased risk of developing endemic Kaposi sarcoma.
• Sex. Men and people AMAB are more likely to develop Kaposi sarcoma than women and people assigned female at birth (AFAB).
• Age. Classic Kaposi sarcoma affects people ages 40 to 70.
• Immune deficiency. People with HIV or AIDS and people taking immunosuppressant medication after organ transplantation have a higher risk of developing Kaposi sarcoma.
• Sexual activity. Unprotected sexual activity can increase the risk of infection with HHV-8 and HIV, viruses typically spread through bodily fluids.

What are complications of Kaposi sarcoma?

Kaposi sarcoma complications may include:

• Anemia: Kaposi sarcoma that affects your digestive system can cause internal bleeding that may lead to anemia.
• Second cancer: People with classic Kaposi sarcoma may develop second cancers, including non-Hodgkin lymphoma.

How is Kaposi sarcoma diagnosed?

Healthcare providers use several different tests to diagnose Kaposi sarcoma. Tests may include:

• Biopsy.
• X-rays.
• CT scan.
• PET scan.
• Endoscopy.
• Bronchoscopy.

How is Kaposi sarcoma treated?

Your healthcare provider will consider several factors while developing your treatment plan. These factors might include:

• The type of Kaposi sarcoma you have.
• The number of lesions you have, and where those lesions are located.
• Your overall health.

For example, if you have AIDs-related Kaposi sarcoma, healthcare providers may use highly active antiretroviral therapy (HAART). If you have acquired Kaposi sarcoma, your provider may reduce or change immunosuppressant medications. Other treatments may include:

• Topical medications.
• Targeted therapy.
• Radiation therapy.
• Chemotherapy.
• Surgery.
• Cryotherapy.

Can Kaposi sarcoma be prevented?

According to the American Cancer Society, taking steps to avoid HIV could prevent most cases of Kaposi sarcoma.

If you have HIV, highly active antiretroviral therapy (HAART) reduces your risk of developing Kaposi sarcoma and AIDS.

I had an organ transplant. 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.

What can I expect if I have Kaposi sarcoma?

That depends on factors like Kaposi sarcoma type, whether treatment eliminated or reduced lesions and your overall health. For example, HAART for HIV/AIDs often eases Kaposi sarcoma symptoms.

How do 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:

• Quitting smoking.
• Getting regular exercise.
• Eating well so you achieve and stay at a healthy weight that’s right for you.
• Avoid or limit the use of beverages containing alcohol. The American Medical Association recommends two drinks a day for men and people AMAB and one drink a day for women and people AFAB.
• Making sure you’re up to date on vaccines.
• Getting enough sleep.
• Washing your hands often.
• Managing stress.

When should I see my healthcare provider?

Kaposi sarcoma can come back (recur) after treatment, so you’ll have regular follow-up appointments so your healthcare provider can monitor your overall health and check for signs of new cancer. (If you have Kaposi sarcoma because you have HIV/AIDS or transplanted organs, your regular follow-up appointments will include monitoring for signs of recurrent Kaposi sarcoma.)

When should I go to the emergency room?

Your healthcare provider will discuss what you can expect from treatments and when it makes sense for you to seek immediate medical care.

What questions should I ask my healthcare provider?

Here are some suggestions:

• What type of Kaposi sarcoma do I have?
• What are my treatment options?
• What are treatment side effects?

A note from Cleveland Clinic

Kaposi sarcoma is a rare and complicated illness that affects different people in different ways. For some people, Kaposi sarcoma is a symptom of HIV/AIDS. For others, it’s a complication of medication needed after an organ transplant. Healthcare providers can ease and sometimes eliminate Kaposi sarcoma symptoms. But the condition often comes back. If you have Kaposi sarcoma, your healthcare provider will explain what you can expect, from diagnosis and treatment to living with Kaposi sarcoma.