What is Kaposi sarcoma?
Kaposi sarcoma is a rare cancer that gained widespread attention during the early days of Human Immunodeficiency Virus/Acquired Immune Deficiency (HIV/AIDS) infections. Since then, innovative medical research and treatment significantly reduced the number of Kaposi sarcoma cases.
People with weakened immune systems are susceptible to Kaposi sarcoma. That said, people only develop Kaposi sarcoma if they are infected with human herpesvirus 8 (HHV-8). HHV-8 is a rare virus. Less than 10% of people in the United States carry HHV-8. This virus is believed to be transmitted via saliva.
Kaposi sarcoma’s most common visible symptom is dark-colored flat or bumpy blotches or patches that appear on a person's arms, legs and face. The patches or blotches, called lesions, can be blue, black, pink, red or purple. Lesions might also appear in a person's mouth, nose and throat. Lesions can spread to internal organs such as the liver, lungs, stomach and the digestive tract.
Is this a common cancer?
Kaposi sarcoma is rare. In the United States, Kaposi sarcoma most frequently appears in people who have AIDS/HIV. Currently, Kaposi sarcoma appears in about six cases per one million people who have AIDS/HIV.
Kaposi sarcoma can also appear in people taking immunosuppressant medication after undergoing organ or bone marrow transplant. About one in 200 people who receive transplants develop Kaposi sarcoma.
Who is affected by Kaposi sarcoma?
Kaposi sarcoma most frequently appears in the following groups of people:
- People who have AIDS/HIV.
- People taking immunosuppressant medication.
- Older people of Mediterranean, Middle Eastern and Eastern European descent and people living in equatorial Africa. More men than women with these backgrounds develop Kaposi sarcoma.
Is this a serious illness?
Kaposi sarcoma can become a serious illness if it spreads to internal organs.
I’ve read that Kaposi sarcoma is a symptom of AIDS or being HIV positive. If I have Kaposi sarcoma, does that mean I have AIDS or am HIV-positive?
While it’s true that Kaposi sarcoma is a symptom of AIDS/HIV, there are other reasons why someone might have Kaposi sarcoma, such as age, ethnicity or taking medication to suppress the immune system.
I found a dark-colored bumpy patch on my leg. Could it be Kaposi sarcoma?
There’s lots of reasons why you might have that blotch. You might have a bruise after bumping into an object. If a bruise hurts and/or lasts for two weeks, talk to your healthcare provider about your concern.
Does everyone infected with HHV-8 develop Kaposi sarcoma?
Most of these infections don’t lead to Kaposi sarcoma. The people at greater risk are those with weak immune systems who are also infected with the virus.
Are there different types of Kaposi sarcoma?
There are four types of Kaposi sarcoma:
- Epidemic Kaposi sarcoma: This is the most common type of Kaposi sarcoma in the U.S. It occurs in six cases per one million people who have HIV/AIDS. Someone who has epidemic Kaposi sarcoma is likely to develop cancerous lesions throughout the body.
- Acquired Kaposi sarcoma: This sarcoma type, sometimes called transplant-related sarcoma, appears in people who have HHV-8 and are undergoing organ or bone transplant that requires medication to suppress their immune system. Acquired Kaposi sarcoma is relatively rare, affecting about one in 200 people undergoing 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’s usually diagnosed in older men of Mediterranean, Middle Eastern and Eastern European descent. People who have classic Kaposi 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 is found in people living in equatorial Africa. This sarcoma is similar to classic Kaposi sarcoma. The difference is age of diagnosis — people generally develop endemic Kaposi sarcoma at a much younger age than those who have classic Kaposi sarcoma.
Symptoms and Causes
What causes Kaposi sarcoma?
Kaposi sarcoma develops when a rare virus called human herpesvirus 8 (HHV-8) infects the cells that line blood and lymphatic vessels. Fewer than 10% of people in the United States have HHV-8. Most HHV-8 infections don’t lead to Kaposi sarcoma.
The virus triggers Kaposi sarcoma by causing normal cells to turn into cancer cells that create lesions.
The people at greater risk for this cancer are infected with HHV-8 and have weak immune systems, either because they have Human Immunodeficiency Virus/Acquired Immune Deficiency (HIV/AIDS) or are taking medication to suppress their immune systems.
What are typical Kaposi sarcoma symptoms?
There are four types of Kaposi sarcoma. While each type’s symptoms vary, one common symptom is dark-colored lesions that appear on a person's face, legs and trunk, and at times in the groin area.
The lesions are unsightly but rarely painful. That said, lesions on a person's legs or in the groin area can cause painful swelling.
What are typical symptoms for someone who has HIV/AIDS?
Someone with suspected or diagnosed HIV or AIDS might experience the following symptoms, which are caused by lesions growing inside the body:
- Dark-colored lesions that appear on the skin and/or lining of the mouth.
- Swelling, known as lymphedema, which happens when a lesion blocks a part of the lymph system.
- Unexplained chest pain or cough.
- Unexplained stomach or intestinal pain.
- Diarrhea or blockage in the digestive tract.
What are the typical symptoms for someone with acquired Kaposi sarcoma?
Skin lesions are the most common symptom for someone with acquired Kaposi sarcoma. In some cases, lesions can spread to the mucous membranes (the inside of the mouth or nose) or other organs in the body.
What are typical symptoms for someone with classic or Mediterranean Kaposi sarcoma?
Typical symptoms include slow-growing dark-colored lesions that appear on the lower body, including legs, ankles or soles of feet.
Lesions might develop in the stomach, intestines, digestive tract or lymph nodes and grow in size and number over a period of 10 or more years.
What are typical symptoms for someone with endemic (African) Kaposi sarcoma?
Skin lesions are the most common symptom for someone with endemic Kaposi sarcoma. In some cases involving children, symptoms include signs of internal lesions. Those symptoms can include:
- Swelling, known as lymphedema, which happens when lesions block part of the lymph system.
- Unexplained chest pain or cough
- Unexplained stomach or intestinal pain.
- Diarrhea or blockage in the digestive tract.
Diagnosis and Tests
How is Kaposi sarcoma diagnosed?
Healthcare providers use a variety of tests to diagnose Kaposi sarcoma, but not all of tests available will be used with every person. Regardless of the test used, healthcare providers will try to make the tests as comfortable as possible.
How does a healthcare provider decide which tests to use?
Healthcare providers consider several factors when deciding which tests to use to make a diagnosis. These factors might include a person's age, general health and symptoms.
What kinds of tests do healthcare providers use?
Your healthcare provider will start by doing a thorough physical examination. They might recommend other tests including:
- Biopsy: A biopsy for suspected Kaposi sarcoma involves removing small amounts of tissue from lesions. Biopsies are the most effective way to diagnose Kaposi sarcoma.
- X-rays: Some Kaposi sarcoma symptoms indicate internal lesions. An X-ray can provide a picture of the body’s internal structures. This picture might reveal internal lesions.
- Computed tomography (CT or CAT) scans: These scans create detailed images that can reveal lesions or tumors. Healthcare providers use CT/CAT scans to measure the size of a lesion.
- Endoscopy: This test is used to inspect the stomach and bowel for signs of Kaposi sarcoma. To do this, healthcare providers insert a thin tube called an endoscope into the mouth, through the esophagus and then down into stomach. People receiving this test are given medicate that will help them relax during the test.
- Bronchoscopy: This tests helps find lesions in the lungs. Healthcare providers pass a thin, flexible tube into the mouth or nose and down through the windpipe to the breathing passages of the lungs. The tube has a light on the end that enables healthcare providers to see inside the lungs and to take fluid and tissue samples. People receiving this test are given mild anesthesia.
- Photography: Healthcare providers might take pictures of the skin over a period of time. This process is called mapping and helps determine if there are new lesions.
I’m really anxious about the tests my healthcare provider recommends. What can I do to feel less stressed?
The best thing you can do is share your concerns with your healthcare provider. They can explain what will happen, including how they will help you deal with any discomfort.
Management and Treatment
I’ve been diagnosed with Kaposi sarcoma. How will my healthcare provider decide which treatments they will use?
Your healthcare provider will consider several factors while developing your treatment plan. These factors might include:
- Your overall health.
- The type of Kaposi sarcoma you have.
- The number of lesions you have, and where those lesions are located.
- How you’re affected, including the kinds of problems you’re having as a result of your illness.
How do healthcare providers treat Kaposi sarcoma?
Healthcare providers treat each type of Kaposi sarcoma in different ways. There are many ways to treat this cancer. For example, acquired Kaposi sarcoma treatment might simply be eliminating the immunosuppressant drugs that cause people to develop lesions.
What is the treatment for epidemic Kaposi sarcoma?
Healthcare providers typically use a combination of anti-AIDS drugs to treat someone who has Kaposi sarcoma Kaposi and Human Immunodeficiency Virus/Acquired Immune Deficiency (HIV/AIDS). Treating the underlying AIDS/HIV infection can shrink lesions. Other options include:
- Highly active antiretroviral therapy or HAART.
What is the treatment for classic or Mediterranean Kaposi sarcoma?
Treatments might include:
- Cryosurgery, which uses extremely cold chemical to treat lesions.
- Electrocoagulation, where providers treat lesions using heat from an electric current.
What are treatments for endemic Kaposi sarcoma?
Typical treatments are:
- Radiation therapy.
Outlook / Prognosis
What is the outlook or prognosis for Kaposi sarcoma?
The prognosis for Kaposi sarcoma depends on the sarcoma type. Below are prognoses for the four types of Kaposi sarcoma:
- Epidemic Kaposi sarcoma: This sarcoma type stems from underlying AIDS/HIV infections. That means the prognosis or outcome depends on successfully treating the underlying infections. Recent studies show antiretroviral therapies reduce the number of Kaposi sarcoma cases in people who have AIDS/HIV.
- Acquired Kaposi sarcoma: These are cases where people undergoing organ or bone marrow transplants develop Kaposi sarcoma because they take medication that suppresses the immune system and helps the transplant process. For these people, changing or eliminating medication might cause the lesions to go away without additional treatment.
- Classic or Mediterranean Kaposi sarcoma: Several factors affect the prognosis for classic Kaposi sarcoma. People who have many lesions, many areas with lesions or internal lesions might have recurring Kaposi sarcoma.
- Endemic or African Kaposi sarcoma: The prognosis for people living in Africa relies on access to treatment.
What kind of follow-up care should I expect?
Follow-up care is essential to see if Kaposi sarcoma has come back, if more treatment is appropriate and to check on any treatment side effects that you might have. During follow up visits, healthcare providers might ask about symptoms and examine you. They might recommend tests including blood tests, X-rays or CT scans.
This is a good time for you and your healthcare team to discuss any changes, concerns or problems you encounter while living with Kaposi sarcoma.
A follow-up appointment is a good time to share any changes, problems or concerns you might have, including concerns about side effects.
What can I do to help myself?
The best thing you can do for yourself is to maintain a healthy immune system. Healthy behaviors such as exercising regularly, eating well to stay at a healthy weight and not smoking can contribute to your overall wellbeing and health.
A note from the Cleveland Clinic
Kaposi sarcoma is a rare and complicated illness that affects different people in different ways. Taking in all the information about Kaposi sarcoma might be overwhelming. Talk to your healthcare provider about your individual diagnosis and what it means for you. Discussing treatment options and the outlook for your condition might help you to feel less overwhelmed. Ask your healthcare provider about resources to help you cope. There are healthcare providers who specialize in managing the stress that a serious illness can bring. There are also support groups where you can connect with people who know what you’re going through. While Kaposi sarcoma is a serious illness, there are several steps healthcare providers can take to ease symptoms and in some cases eliminate symptoms. Research on Kaposi sarcoma is helping develop even more effective treatments.
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