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What is Kaposi sarcoma?
Kaposi sarcoma (KS) is a cancer that develops from the cells that line lymph or blood vessels. It usually appears as tumors on the skin or on mucosal surfaces such as inside the mouth, but tumors can also develop in other parts of the body, such as in the lymph nodes (bean-sized collections of immune cells throughout the body) or digestive tract.
The abnormal cells of KS form purple, red, or brown blotches or tumors on the skin. These affected areas are calledlesions. The skin lesions of KS most often appear on the legs or face. They may look bad, but they usually cause no symptoms. Some lesions on the legs or in the groin area may cause the legs and feet to swell painfully.
KS can cause serious problems or even become life threatening when the lesions are in the lungs, liver, or digestive tract. KS in the digestive tract, for example, can cause bleeding, while tumors in the lungs may cause trouble breathing.
Types of Kaposi sarcoma
The different types of KS are defined by the different populations it develops in, but the changes within the KS cells are very similar.
Epidemic (AIDS-related) Kaposi sarcoma
The most common type of KS in the United States is epidemic or AIDS-related KS. This type of KS develops in people who are infected with HIV, the virus that causes AIDS.
A person infected with HIV (that is, who is HIV-positive) does not necessarily have AIDS. The virus can be present in the body for a long time, often many years, before causing major illness. The disease known as AIDS begins when the virus has seriously damaged the immune system, which results in certain types of infections or other medical complications, including KS. When HIV damages the immune system, people who also are infected with a certain virus (the Kaposi sarcoma herpesvirus or KSHV) are more likely to develop KS.
KS is considered an “AIDS defining” illness. This means that when KS occurs in someone infected with HIV, that person officially has AIDS (and is not just HIV-positive).
In the United States, treating HIV infection with highly active antiretroviral therapy (HAART) has resulted in fewer cases of epidemic KS. It can often keep advanced KS from developing. HAART doesn’t completely protect against KS; it can still occur in people whose HIV is well controlled with HAART. Once KS develops it is still important to continue HAART.
In areas of the world where HAART is not easy to obtain, KS in AIDS patients can advance quickly.
Classic (Mediterranean) Kaposi sarcoma
Classic KS occurs mainly in older people of Mediterranean, Eastern European, and Middle Eastern heritage. Classic KS is more common in men than in women.
Patients typically have one or more lesions on the legs, ankles, or the soles of the feet. Compared to other types of KS, the lesions in this type do not grow as quickly, and new lesions do not develop as often.
The immune system of people with classic KS is not as weak as it is in those who have epidemic KS, but it may be weaker than normal. Getting older can naturally weaken the immune system a little. When this occurs, people who already have a KSHV infection are more likely to develop KS.
Endemic (African) Kaposi sarcoma
Endemic KS occurs in people living in Equatorial Africa and is sometimes called African KS. KSHV infection is much more common in Africa than in other parts of the world, so the risk of KS is higher. Other factors in Africa also probably contribute to the development of KS, since the disease affects a broader group of people that includes children and women.
Endemic KS tends to occur in younger people (usually under age 40). Rarely a more aggressive form of endemic KS is seen in children before puberty. This type usually affects the lymph nodes and other organs and can progress quickly.
Endemic KS used to be the most common type of KS in Africa. Then, as AIDS became more common in Africa, the epidemic type became more common.
Iatrogenic (transplant-related) Kaposi sarcoma
When KS develops in people whose immune systems have been suppressed after an organ transplant, it is callediatrogenic, or transplant-related KS. Most transplant patients need to take drugs to keep their immune system from rejecting (attacking) the new organ. But by weakening the body’s immune system, these drugs increase the chance that someone infected with KSHV will develop KS. Stopping the immune-suppressing drugs or lowering their dose often makes KS lesions go away or get smaller.
Kaposi sarcoma in HIV negative men who have sex with men
Recently, there have been reports of KS developing in men who have sex with men who are not infected with HIV. In this group, the cases of KS are often mild, similar to cases of classic KS.
Do we know what causes Kaposi sarcoma?
Kaposi sarcoma (KS) is caused by infection with a virus called the Kaposi sarcoma herpesvirus (KSHV), also known as human herpesvirus 8 (HHV8). KSHV is similar to 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.
Can Kaposi sarcoma be prevented?
Kaposi sarcoma (KS) is caused by the Kaposi sarcoma 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.
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 infections, not to prevent KS.
Can Kaposi sarcoma be found early?
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 should see their doctors right away so that the cause can be found as soon as possible and treated, if needed.
How is Kaposi sarcoma diagnosed?
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.
Signs and symptoms of Kaposi sarcoma
KS usually appears first on the skin, forming purple, red, or brown patches (not raised above the surrounding skin), plaques (flat areas that are slightly raised), or nodules (bumps) that are called lesions. The skin lesions of KS most often develop on the legs or face, but they can also appear in other areas. Lesions can also develop on mucous membranes such as those inside the mouth. The lesions are usually not painful or itchy. Some lesions on the legs or in the groin area might block the flow of fluid out of the legs. This can lead to painful swelling in the legs and feet.
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 intestines can cause abdominal pain, diarrhea, or bleeding from the rectum.
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.
Chest x-ray
The 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.
If someone is 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 the patient is 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, the patient is 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) is used to look at the inner lining of the esophagus, the stomach, and the first part of the small intestine. For this procedure, the patient is first given drugs to make them 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 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. Sometimes enemas are used as well. Just before the procedure, the patient is given intravenous (IV) medicine to make him or her relaxed or even asleep. 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 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, the patient 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 the person’s waist while he or she goes 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.
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. The patient is given intravenous (IV) medicine to make him or her relaxed, or even 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 may take as long as 2 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 if the patient is already known to have KS based on biopsies of other tissues, such as skin, lungs, or intestines.
How is Kaposi sarcoma staged?
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 . 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 patients 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
Survival of patients with Kaposi sarcoma
Survival rates are often used by doctors as a standard way of discussing a person’s prognosis (outlook). Some patients with Kaposi sarcoma (KS) may want to know the survival statistics for people in similar situations, while others may not find the numbers helpful, or may even not want to know them.
The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Of course, many people live much longer than 5 years.
Five-year relative survival rates assume that some people will die of other causes and compare the observed survival with that expected for people without the cancer. This is a better way to see the impact of the cancer on survival.
In order to get 5-year survival rates, doctors have to look at people who were treated at least 5 years ago. Improvements in treatment since then may result in a more favorable outlook for people now being diagnosed with KS.
Survival rates are often based on previous outcomes of large numbers of people who had the disease, but they cannot predict what will happen in any particular person’s case. Many factors affect a person’s outlook, such as where the KS is in the body, the person’s age and general health, how well their immune system functions, and their response to highly active antiretroviral therapy. Your doctor can tell you how the numbers below apply to you, as he or she is familiar with your particular situation.
As treatment of the HIV infection continues to improve, so does the outlook for people with KS. It takes time to see the effect of the most up-to-date treatment on survival rates, since they are based on patients first diagnosed many years ago. Early in the AIDS epidemic, the outlook for patients with KS was grim, with less than 10% of patients surviving at least 5 years after diagnosis. This has improved over time, with the most recent data from the National Cancer Institute’s SEER program showing an overall 5-year relative survival of about 68%. The cause of death for people with KS is not always the KS. Often, people with KS die from diseases related to HIV and AIDS, and not the KS itself.
Research has shown that people who are in good risk groups in the AIDS Clinical Trials Group (ACTG) system have better survival rates than those who are in the poor risk group.
