Prevention

 

Kaposi sarcoma (KS) is caused by the Kaposi sarcoma--associated herpesvirus (KSHV). There are no vaccines at this time to protect people against KSHV. For now, preventing KS depends on reducing the chance of becoming infected with KSHV and reducing the chance that people who are infected with KSHV will develop KS.

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Most cases of KS occur in people with HIV and AIDS. Taking measures to avoid becoming infected with HIV could prevent most cases of KS.

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• Since HIV can be spread through sex, avoiding unprotected sex with people infected with HIV could help prevent these infections. Many people with HIV don’t know that they are infected, so many public health workers recommend using a condom during any sexual contact.

• Another way to lower the risk of getting HIV is to take a pill every day that contains ant-viral drugs. This is called pre-exposure prophylaxis (PrEP). At this time, the Centers for Disease Control and Prevention (CDC) recommends PrEP for people who are HIV negative and at very high risk for HIV. The CDC has more information about who should use PrEP.

• HIV can also be spread through the use of contaminated (dirty) needles to inject recreational drugs. For people who inject drugs, the safest way to avoid HIV is to quit. However, some people are unable to quit on their own or get help in quitting, and they may not be able to stop using drugs right away. For these people, clean needles and injection supplies can help protect them. In some areas, there are programs to make sure that drug users can get clean needles and syringes.

• HIV-infected mothers can pass the virus to their babies during pregnancy, delivery, or breastfeeding. Treating the mothers and infants with anti-HIV drugs and avoiding breastfeeding can greatly reduce the risk of these infections.

• In the past, blood product transfusions and organ transplants were responsible for some HIV infections. As a result of improved testing for HIV, there is now a very low risk of HIV infection from blood products or organ transplants.

 

For people who are infected with HIV and KSHV, taking the right medicines can reduce the chance of developing KS.

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• Testing for HIV can identify people infected with this virus. People with HIV should get treatment to help strengthen their immune system, which usually includes highly active antiretroviral therapy (HAART). HAART reduces the risk that people with HIV will develop KS (and AIDS). Treating infections that commonly occur in people with weakened immunity also reduces the likelihood of developing problems with KS.

• HIV-infected people who take drugs to treat herpesvirus infections (such as ganciclovir or foscarnet) are less likely to develop KS because these drugs also work against KSHV (which is a type of herpesvirus). Still, these drugs can have serious side effects, so they are only taken to treat certain viral infections, not to prevent KS

 

For people who are at risk of developing KS after an organ transplant (iatrogenic KS), using certain types of immune suppressive drugs, such as sirolimus or everolimus (mTOR inhibitors), may reduce the chances of KS while still helping prevent rejection of the new organ.

 

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Early Detection

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Most cancers start in one place and then spread to other parts of the body. When these cancers are found early, they are more likely to be curable. Kaposi sarcoma (KS) is different, because it tends to form in several areas at the same time. Even when only one skin lesion is visible, many people already have other areas of KS that are just too small to be seen.

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There are no recommended routine screening tests to look for KS in people who are not at increased risk of the disease.

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People infected with HIV are much more likely to develop KS, so many health experts recommend that people infected with HIV be examined regularly by health care providers who are experienced in recognizing KS and other diseases that go along with HIV infection and AIDS. People with possible symptoms of KS should see their doctors right away so that the cause can be found as soon as possible and treated, if needed.

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Signs and Symptoms 

 

Kaposi sarcoma (KS) usually appears first as spots (called lesions) on the skin. The lesions can be purple, red, or brown. KS lesions can be flat and not raised above the surrounding skin (called patches), flat but slightly raised (called plaques), or bumps (called nodules). The skin lesions of KS most often develop on the legs or face, but they can also appear in other areas. Lesions on the legs or in the groin area can sometimes block the flow of fluid out of the legs. This can lead to painful swelling in the legs and feet.

 

KS lesions can also develop on mucous membranes (the inner linings of certain parts of the body) such as inside the mouth and throat and on the outside of the eye and inner part of the eyelids. The lesions are usually not painful or itchy.

 

KS lesions can also sometimes appear in other parts of the body. Lesions in the lungs might block part of an airway and cause shortness of breath. Lesions that develop in the stomach and intestines can cause abdominal pain and diarrhea.

 

Sometimes KS lesions bleed. If the lesions are in the lung, it can cause you to cough up blood and lead to shortness of breath. If the lesions are in the stomach or intestines, it can cause bowel movements to become black and tarry or bloody. Bleeding from lesions in the stomach and intestines can be so slow that blood isn’t visible in the stool, but over time the blood loss can lead to low red blood cell counts (anemia). This can cause symptoms like tiredness and shortness of breath.

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Tests 

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Kaposi sarcoma (KS) is often found when a person goes to the doctor because of signs or symptoms they are having. Sometimes KS may be found during a routine physical exam. If KS is suspected, further tests will be needed to confirm the diagnosis.

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Medical history and physical exam

If your doctor suspects you might have KS, you will be asked about your medical history to learn about illnesses, operations, your sexual activity, and other possible exposures to Kaposi sarcoma--associated herpesvirus (KSHV) and HIV. The doctor will ask you about your symptoms and about any skin tumors you have noticed.

 

As part of a complete physical exam, the doctor will examine your skin and the inside of your mouth to look for KS lesions. Sometimes KS lesions develop inside the rectum (the part of the large intestine just inside the anus). A doctor might be able to feel these lesions during an exam with a gloved finger. The doctor may also check the stool for occult (unseen) blood, since KS in the intestines can cause bleeding.

 

Biopsy

To be sure that a lesion is caused by KS, the doctor will need to take a small sample of tissue from the lesion and send it to a lab to be checked. This is called a biopsy. A specially trained doctor called a pathologist can often diagnose KS by looking at the cells in the biopsy sample in the lab.

 

For skin lesions, the doctor will usually perform a punch biopsy, which removes a tiny round piece of tissue. If the entire lesion is removed, it is called an excisional biopsy. These procedures can often be done with just local anesthesia (numbing medicine).

 

Lesions in other areas, such as the lungs or intestines, can be biopsied during other procedures such as bronchoscopy or endoscopy, which are described below. Since biopsy of lesions in these areas can sometimes cause serious bleeding, biopsy is often not done in people already known to have KS.

 

Chest X-ray

Your lungs may be x-rayed to see if KS is there. If the x-ray shows something abnormal, other tests, such as a CT scan, might be needed to tell for sure if it is KS or some other condition.

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For people known to have KS in the lung, chest x-rays can be used to see how the disease is responding to treatment.

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Bronchoscopy

Bronchoscopy is a test that lets the doctor look into the windpipe (trachea) and the large airways of the lungs. This procedure is often done if you are having problems such as shortness of breath or coughing up blood, or if the chest x-ray or CT scan shows something abnormal. Any of these could mean that KS is in the lungs.

 

Before bronchoscopy starts, you are put to sleep with a light anesthesia. Then the doctor inserts the bronchoscope (a thin, flexible lighted tube with a small video camera on the end) through the mouth, down the windpipe, and into the lungs. If the doctor sees an abnormal area that might be KS, it can be biopsied through the bronchoscope. Bronchoscopy with biopsies can also be used to help diagnose other lung problems seen in AIDS patients, such as pneumonia.

 

Gastrointestinal endoscopy

One or more of these tests might be done when the doctor suspects that KS is in the stomach or intestines and is causing problems.

 

Upper endoscopy (also called esophagogastroduodenoscopy, or EGD)

Upper endoscopy is used to look at the inner lining of the esophagus, the stomach, and the first part of the small intestine. For this procedure, you are first given drugs to make you sleepy. Then, the doctor guides the endoscope (a thin, flexible, lighted tube with a small video camera on the end) through the mouth and esophagus and into the stomach and small intestine. This lets the doctor see things like ulcers, infections, and KS lesions.

 

If an abnormal area is seen, the doctor can use small surgical instruments through the endoscope to biopsy it.

 

Colonoscopy

Colonoscopy is used to look inside the large intestine (colon and rectum). Before this test can be done, the colon and rectum must be cleaned out to remove any stool. This often means drinking a large amount of a liquid laxative the night before and the morning of the procedure, and spending a lot of time in the bathroom.

 

Just before the procedure, you will be given intravenous (IV) medicine to make you relaxed or even asleep (sedation). Then a colonoscope (a long, flexible, tube with a light and video camera on the end) is inserted through the rectum and into the colon. Any abnormal areas seen can be biopsied.

 

Capsule endoscopy

Capsule endoscopy is a way to look at the small intestine. It is not truly a type of endoscopy, since it doesn’t use an endoscope. Instead, you swallow a capsule (about the size of a large vitamin pill) that contains a light source and a very small camera. Like any other pill, the capsule goes through the stomach and into the small intestine.

 

As it travels through the small intestine (usually over about 8 hours), it takes thousands of pictures. These images are transmitted electronically to a device worn around your waist while you go on with normal daily activities. The images can then be downloaded onto a computer, where the doctor can look at them as a video.

 

The capsule passes out of the body through the stool during a normal bowel movement and is discarded. A disadvantage of this test is that it doesn’t allow the doctor to biopsy any abnormal areas. You will probably be told not to eat or drink for about 12 hours before the test.

 

Double balloon enteroscopy

Double balloon enteroscopy is another way to look at the small intestine. Regular endoscopy cannot look very far into the small intestine because it is too long and has too many curves. This method gets around these problems by using a special endoscope that is made up of 2 tubes, one inside the other. For this test, you are given intravenous (IV) medicine to make you relaxed (sedation), and may be even given general anesthesia (so that you are asleep).

 

The endoscope is then inserted either through the mouth or the anus, depending on if there is a specific part of the small intestine to be examined. Once inside the small intestine, the inner tube, which has the camera on the end, is advanced about a foot as the doctor looks at the lining of the intestine. Then a balloon at its end is inflated to anchor it. The outer tube is then pushed forward to near the end of the inner tube and is anchored in place with a second balloon.

 

This process is repeated over and over, letting the doctor see the intestine a foot at a time. The doctor can even take a biopsy if something abnormal is seen. This procedure is more involved than capsule endoscopy (and can take hours to complete), but it has the advantage of letting the doctor biopsy any lesions seen.

 

KS can also affect other organs, such as the liver, spleen, heart, or bone marrow. These areas do not often need to be biopsied in people already known to have KS based on biopsies of other tissues, such as skin, lungs, or intestines.