Before the AIDS epidemic, Kaposi sarcoma (KS) was rare in the United States. At that time, only about 2 new cases of KS were found for every million people in the United States each year. Most often, the types of KS that occurred were classic and transplant-related.

With the AIDS epidemic, the rate of KS in this country increased more than 20 times — peaking at about 47 cases per million people (per year) in the early 1990s. Early in the AIDS epidemic, patients infected with HIV in the United States were estimated to have a 1 in 2 chance of developing KS.

With new treatments for AIDS, KS has become less common in the United States, and it now occurs at a rate of about 6 cases per million people each year. It is still seen most often in people infected with HIV. In the United States, KS is much more common in men than in women, and it is rarely seen in children. It is also more common in African Americans than in whites in the United States. Transplant recipients are another group that gets KS. About 1 in 200 transplant patients in the United States gets KS. Most of these people were already infected with Kaposi sarcoma associated herpesvirus (KSHV) before the transplant, but the virus didn’t cause problems because their immune system kept it in check. The drugs these people take to suppress their immune system allow KS to develop.

In areas of the world (such as Africa) where KSHV and HIV infection rates are high, both endemic and HIV-associated KS are seen, and KS occurs in men, women, and children.

Visit the American Cancer Society’s Cancer Statistics Center for more key statistics.

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.

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 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 analyzed. 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 under a microscope.

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 might be needed to tell for sure if it is KS or some other condition.

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

Bronchoscopy

Bronchoscopy 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 shows something abnormal. Any of these could mean that KS is in the lungs.

Before starting the bronchoscopy, 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.

Because anesthesia is used, you will need someone you know to take you home (not just a cab).

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. Because sedation is used for this procedure, you will need someone you know to help you get home afterward (not just a cab).

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 (2 to 4 quarts) 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 go to sleep (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. Because sedation is used for this procedure, you will need someone you know to help you get home afterward (not just a cab).

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 swallows 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 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 likely 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. Because sedation (or general anesthesia is used), you will need someone you know to take you home (not just a cab).

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.

For many types of cancer, the stage is a description of how far the cancer has spread, based on the results of physical exams, biopsies, and imaging or other tests (see  How is Kaposi Sarcoma Diagnosed?). The stage of a cancer is often one of the most important factors in selecting treatment options and predicting a patient’s survival outlook.

The results of the staging process are usually described in a standard way, using a staging system. Staging systems for most other types of cancer are based on the size of the primary tumor (the first one to develop) and how far the cancer has spread from there. But for people with AIDS-related Kaposi sarcoma (KS), the most common type in the United States, the outlook is influenced at least as much by the presence of other AIDS-related problems as it is by the spread of KS. For this reason, staging of KS also considers factors such as how much the immune system is damaged and the presence of AIDS-related infections.

There is no officially accepted system for staging KS like there is for most other forms of cancer. But for AIDS-related KS, most doctors use the AIDS Clinical Trials Group system.

The AIDS Clinical Trial Group system

The AIDS Clinical Trials Group (ACTG) system for AIDS-related KS considers 3 factors:

• The extent of the tumor (T)
• The status of the immune system (I), as measured by the number of certain immune cells (CD4 cells) present in the blood
• The extent of involvement within the body or systemic illness (S)

Under each major heading, there are 2 subgroups: either a 0 (good risk) or a 1 (poor risk). The following are the possible staging groups under this system:

T (tumor) status

T0 (good risk): Localized tumor

KS is only in the skin and/or the lymph nodes (bean-sized collections of immune cells throughout the body), and/or there is only a small amount of disease on the palate (roof of the mouth). The KS lesions in the mouth are flat rather than raised.

T1 (poor risk): The KS lesions are widespread. One or more of the following is present:

• Edema (swelling) or ulceration (breaks in the skin) due to the tumor
• Extensive oral KS: lesions that are nodular (raised) and/or lesions in areas of the mouth besides the palate (roof of the mouth)
• Lesions of KS are in organs other than lymph nodes (such as the lungs, the intestine, the liver, etc.). Kaposi sarcoma in the lungs is a particularly bad sign.

I (immune system) status

The immune status is assessed using a blood test known as the CD4 count, which measures the number of white blood cells called helper T cells.

I0 (good risk): CD4 cell count is 150 or more cells per cubic mm (mm³).

I1 (poor risk): CD4 cell count is lower than 150 cells per mm³.

S (systemic illness) status

S0 (good risk): No systemic illness present; all of the following are true:

No history of opportunistic infections (infections that rarely cause problems in healthy people but affect people with suppressed immune systems) or thrush (a fungal infection in the mouth).

No B symptoms lasting more than 2 weeks. B symptoms include:

• Unexplained fever
• Night sweats (severe enough to soak the bed clothes)
• Weight loss of more than 10% without dieting
• Diarrhea

And this is true:

• Karnofsky performance status (KPS) score of 70 or higher. This means you are up and about most of the time and able to take care of yourself.

S1 (poor risk): Systemic illness present; one or more of the following is true:

• History of opportunistic infections or thrush
• One or more B symptoms is present
• KPS score is under 70
• Other HIV-related illness is present, such as neurological (nervous system) disease or lymphoma

Overall risk group

Once these features have been assessed, patients are assigned an overall risk group (either good risk or poor risk). In fact, since highly active antiretroviral therapy (HAART) became available to treat HIV, the immune status (I) has become less important and is often not counted in determining the risk group:

• Good risk: T0 S0, T1 S0, or T0 S1
• Poor risk: T1 S1

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 linings of certain parts inside 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.

Kaposi sarcoma (KS) is caused by infection with a virus called the Kaposi sarcoma associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV8). KSHV is in the same family as Epstein-Barr virus (EBV), the virus that causes infectious mononucleosis (mono) and is linked to several types of cancer.

In KS, the cells that line blood and lymphatic vessels (called endothelial cells) are infected with KSHV. The virus brings genes into the cells that can cause the cells to divide too much and to live longer than they should. These types of changes may eventually turn them into cancer cells.

KSHV infection is much more common than KS, and most people infected with this virus do not get KS. Many people infected with KSHV will never show any symptoms. Infection with KSHV seems to be needed to cause KS, but in most cases infection with KSHV alone does not lead to KS. Most people who develop KS also have a weakened immune system, due to HIV infection, organ transplant, older age, or some other factor.

The percentage of people infected with KSHV varies in different places around the world. In the United States, studies have found that less than 10% of people are infected with KSHV. The infection is more common in people infected with HIV than in the general population in the United States. KSHV is more common in men who have sex with men than in men who only have sex with women.

In some areas of Africa, more than 90% of the population shows signs of KSHV infection. In these areas the virus seems to spread from mother to child. KSHV is also found in saliva, which may be one of the ways it is passed to others.

For more on infections and their role in cancer, see Infections That Can Lead to Cancer.

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.

Most cases of KS in the United States occur in people with AIDS. Taking measures to avoid becoming infected with HIV could prevent most cases of KS in this country.

• 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. (A condom may not be needed if both people are HIV-negative and are in a mutually monogamous relationship). Abstinence is the most effective protection.
• 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 substantial risk for HIV.” The CDC has more information about who should use PrEP at www.cdc.gov/hiv/prevention/research/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 in the United States.

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

• 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.

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.

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

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 (see  Signs and Symptoms of Kaposi Sarcoma) should see their doctors right away so that the cause can be found as soon as possible and treated, if needed.

Treatment for Kaposi sarcoma (KS) can often help control the disease or even make the lesions go away, but there is no guarantee that KS will not come back. It is important to be aware that there is always a chance that KS lesions will return, either at the original site or in other places on the skin or internal organs. Advanced KS usually means a person has advanced AIDS, with all its problems. Still, treatment for KS is very effective at taking care of the symptoms of the disease. It can shrink or get rid of bulky, painful lesions and can relieve swelling. It can also reduce problems that arise when tumors affect the lungs or gastrointestinal tract. Treatment for KS can bring about important improvements in your quality of life.

If cancer keeps growing or comes back after one kind of treatment, it is possible that another treatment plan might at least shrink it enough to help you live longer and feel better. But when a person has tried many different treatments and the cancer is not getting any better, it tends to become resistant to all treatment. If this happens, it’s important to weigh the possible limited benefits of a new treatment against the possible downsides. Everyone has their own way of looking at this.

This is likely to be the hardest part of your battle with cancer — when you have been through many medical treatments and nothing’s working anymore. Your doctor may offer you new options, but at some point you might need to consider that treatment is not likely to improve your health or change your outcome or survival.

If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. In many cases, your doctor can estimate how likely it is the cancer will respond to treatment you are considering. For instance, the doctor may say that more treatment might have about a 1 in 100 chance of working. Some people are still tempted to try this. But it is important to think about and understand your reasons for choosing this plan.

No matter what you decide to do, you need to feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care.

Palliative care helps relieve symptoms, but is not expected to cure the disease. It can be given along with cancer treatment, or can even be a cancer treatment. The difference is its purpose — the main goal of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or nausea. Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve pain caused by the cancer. Or chemo might be used to help shrink a tumor and keep it from blocking the bowels. But this is not the same as treatment to try to cure the cancer.

At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn’t mean you can’t be treated for the problems caused by your cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time. You can learn more about hospice and this phase of cancer in Hospice Care and Nearing the End of Life.

Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still hope for good times with family and friends — times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you’ve always wanted to do and to stop doing the things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.

For some people with Kaposi sarcoma (KS), treatment may completely remove or destroy the cancer. Completing treatment can be both stressful and exciting. You will be relieved to finish treatment, yet it is hard not to worry about cancer coming back. (When cancer returns, it is called recurrence.) This is a very real concern for those who have KS, since treatments often do not cure the disease completely.

It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are leading full lives. See Understanding Recurrence for more detailed information on this.

For many people with KS, the cancer never goes away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty. See Managing Cancer As a Chronic Illness for more about this.

Follow-up care

Even if your treatment ends, your doctors will still want to watch you closely. It is very important to go to all of your follow-up appointments. During these visits, your doctors will ask questions about any problems you are having and may do exams and order lab tests or x-rays and scans to look for signs of cancer or treatment side effects. Almost any cancer treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. This is the time for you to talk to your cancer care team about any changes or problems you’ve noticed and any questions or concerns you have.

After your cancer treatment is finished, you will probably need to still see your cancer doctor for many years. Talk with your doctor about what kind of follow-up schedule you can expect.

It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.

Should your cancer come back, see Coping with Cancer Recurrence for information on how to manage this phase of your treatment.

Seeing a new doctor

At some point after your cancer diagnosis and treatment, you may find yourself seeing a new doctor who does not know anything about your medical history. It is important that you be able to give your new doctor the details of your diagnosis and treatment. Gathering these details soon after treatment may be easier than trying to get them at some point in the future. Make sure you have this information handy:

• A copy of your pathology report(s) from any biopsies or surgeries
• Copies of imaging tests (CT or MRI scans, etc.), which can usually be stored on a CD, DVD, etc.
• If you had surgery, a copy of your operative report
• If you stayed in the hospital, a copy of the discharge summary that doctors prepare when patients are sent home
• If you were treated with radiation, a copy of the treatment summary
• If you had drug treatment (including chemotherapy, antiviral drugs, and/or biologic therapy), a list of the drugs, drug doses, and when you took them