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Cancer Medicine :: HIV and AIDS-Related Cancer (Kaposi Sarcoma)Treatment

Kaposi Sarcoma

General treatment information

Treatment for Kaposi sarcoma (KS) is more effective than it was a couple of decades ago. Doctors now better understand what causes KS and have much more experience treating KS than they did when this disease was quite rare. Many clinical trials have compared different approaches to treatment.

Choices about the best treatment options for each patient are based on the function of the immune system as well as the number, location, and size of the KS lesions. The patient’s general health is also a major factor. The presence and severity of other serious health problems can make some treatments a poor choice.

For patients with immune system problems, the most important treatment is keeping the immune system healthy and any related infections under control. Some of the other treatments used for KS are:

Local therapy

Radiation therapy

Chemotherapy

Biologic therapy (immunotherapy)

In some patients, 2 or more of these treatments are used together.

Based on your options, you may have different types of doctors on your treatment team. These doctors may include:

An infectious disease specialist: a doctor who treats infectious diseases such as HIV and AIDS.

A dermatologist: a doctor who treats diseases of the skin

A radiation oncologist: a doctor who treats cancer with radiation therapy.

A medical oncologist: a doctor who treats cancer with medicines such as chemotherapy.

Many other specialists may be involved in your care as well, including physician assistants, nurse practitioners, nurses, nutrition specialists, social workers, and other health professionals.

It is important to discuss all of your treatment options as well as their possible side effects with your doctors to help make the decision that best fits your needs. If time permits, it is often a good idea to seek a second opinion. A second opinion can provide more information and help you feel confident about your chosen treatment plan.

Treating immune deficiency and related infections in people with Kaposi sarcoma

The most important treatment for Kaposi sarcoma (KS) is treating any immune deficiency that exists as well as any related infections.

In people with AIDS, this means using combinations of anti-HIV drugs. This is known as highly active antiretroviral therapy (HAART). For many AIDS patients, HAART may be the only treatment needed to cause the KS lesions to shrink and to keep them under control.

In organ-transplant patients whose immune systems are suppressed by drugs, decreasing or changing the drugs may be helpful.

New KS lesions are more likely to develop when a patient’s blood test results for Kaposi sarcoma herpesvirus (KSHV) are positive. The risk of developing new lesions is lower when antiviral medicines such as ganciclovir or foscarnet are used. These medicines may help prevent new lesions but do not help existing lesions get better.

KS lesions tend to get worse when patients develop bacterial infections. Therefore, it is very important to do what you can to lower your risk of bacterial infections and to treat them promptly if they do occur.

Local therapy for Kaposi sarcoma Local treatment only affects certain Kaposi sarcoma (KS) lesions (or areas of lesions). This type of treatment is often used to treat a few skin lesions in one spot to help a person look or feel better. Local therapy is most useful when there are just a few lesions in a very visible area (such as the face). The drawbacks of local therapy are that it doesn’t treat lesions anywhere else and it can’t keep new lesions from developing.

Topical retinoid treatment: This type of treatment puts medicine directly on the lesion. For example, alitretinoin, a retinoid drug related to vitamin A, is available as a gel (Panretin®) that can be used to treat KS skin lesions. When it is placed on a KS lesion 2 to 4 times a day, it makes it get smaller or go away in 1 to 2 months. Side effects of this gel include skin irritation and lightening of the skin.

Cryosurgery (cryotherapy): Cryosurgery can be useful for small KS lesions on the face, although it is not as helpful for large or deeper lesions. Liquid nitrogen is applied to the tumor to freeze and kill the cells. After the dead area of skin thaws, it may swell, blister and crust over. The wound may take several weeks to heal, and the skin of the treated area may be lighter after treatment.

Surgery: When a person has only a few, small Kaposi sarcoma lesions, one option may be to remove them with surgery. This can be done in different ways.

Simple excision: For this procedure, the skin is first numbed with a local anesthetic. The tumor is then cut out with a surgical knife, along with some surrounding normal skin. The remaining skin is carefully stitched back together, leaving a small scar.

Curettage and electrodesiccation: This treatment removes a tumor by scraping it with a curette (a long, thin instrument with a sharp looped edge on one end), then treating the area with an electric needle (electrode) to try to destroy any remaining cancer cells. This process can be repeated.

A drawback of surgery is that the lesion might recur (come back) in the same place.

Intralesional chemotherapy: For this treatment, a small amount of a chemotherapy drug is injected directly into the KS lesions. Very little of the drug is absorbed into the body. This lets the patient avoid many of the side effects normally seen with chemotherapy.

The most common drug used for intralesional chemotherapy in KS is vinblastine. Sodium tetradecyl sulfate (STS or Sotradecol®) can also be used. Some people may have swelling, blistering, and pain at the injection site with this type of treatment.

Photodynamic therapy (PDT): This treatment uses a special liquid drug that is applied to the skin. The drug collects in the tumor cells over several hours or days and makes the cells sensitive to certain types of light. A special light source is then focused on the tumor(s), and the cells die. A possible side effect of PDT is that it can make the skin very sensitive to sunlight for some time, so precautions may be needed to avoid severe burns.

Radiation therapy for Kaposi sarcoma

Radiation therapy uses high-energy radiation to kill cancer cells. When the radiation is delivered from outside the body it is called external beam radiation therapy. This is the type of radiation therapy used to treat lesions of Kaposi sarcoma (KS).

Radiation therapy is often effective as a type of local therapy to treat KS lesions on or near the surface of the body. Radiation is used to reduce symptoms like pain or swelling. It is also used for skin lesions that look bad and are in places that can easily be seen (like the face).

For KS lesions on the skin, the form of radiation most often used is called electron-beam radiation therapy (EBRT). It uses tiny particles called electrons that don’t penetrate far past the skin’s surface. This limits non-skin side effects. EBRT can also be used to treat large areas of the skin if a person has many widespread KS lesions.

Radiation can also be used to treat KS lesions in the mouth or throat. The form of radiation used for this, known asphoton radiation, can penetrate deeper into the body.

Radiation treatments for KS lesions are often given once a week for several weeks. Getting treatment is much like getting an x-ray, but the radiation is stronger. The procedure itself is painless. Each treatment lasts only a few minutes, although the setup time − getting you into place for treatment − takes longer.

Side effects of radiation therapy can include skin changes, nausea, vomiting, and fatigue, Radiation can also cause anemia (low red blood cells), as well as lower numbers of white blood cells, which increases the risk of infection. Serious side effects are rare when radiation is given to just a small area of the skin, but a small portion of patients have severe skin reactions. When radiation is used to treat KS lesions in the mouth or throat, these areas can become painful and open sores can develop. If chemotherapy and radiation are given at the same time, the side effects are worse.

Chemotherapy for Kaposi sarcoma

Chemotherapy (chemo) is the use of drugs to treat cancer. When the drugs are given into a vein or by mouth, they enter the bloodstream to reach all areas of the body. This is a type of systemic treatment. It is useful to treat cancer that has spread to many areas of the body. When the drugs are injected directly into a tumor it is called intralesional chemotherapy .

The systemic chemo drugs used most often to treat Kaposi sarcoma (KS) belong to a group known as liposomal anthracyclines. Anthracyclines are drugs that treat many different cancers. In liposomal anthracyclines, the drugs are enclosed in tiny fat globules. In this form, they are better taken up by tumors and have fewer side effects. The liposomal anthracyclines used  to treat KS are liposomal doxorubicin (Doxil®) and liposomal daunorubicin(DaunoXome®).

Other chemotherapy drugs that treat KS include paclitaxel (Taxol®), gemcitabine (Gemzar®), and vinorelbine(Navelbine®). Drugs used less often include bleomycin, vinblastine (Velban®), vincristine (Oncovin®), and etoposide(VP-16).

More than half of KS patients treated with chemotherapy will improve, but KS generally doesn’t go away completely. Sometimes chemotherapy can be stopped as long as lesions are not causing problems or increasing in size and number. If the KS starts to get worse, treatment can resume.

It can be hard to give chemotherapy for long periods of time in people who have immune system problems (such as AIDS) because chemo drugs can also weaken the immune system. In all patients, it is important to try to improve immune function and treat related infections. This is especially important when giving chemotherapy.

Chemo drugs attack cells that are dividing quickly, which is why they work against cancer cells. But other cells in the body, such as those in the bone marrow, the lining of the mouth and intestines, and the hair follicles, also divide quickly. These cells are also likely to be affected by chemotherapy, which can lead to side effects such as:

Nausea and vomiting

Loss of appetite

Mouth sores

Diarrhea

Hair loss

Increased risk of infection (from too few white blood cells)

Easy bruising or bleeding (from too few blood platelets)

Fatigue (from too few red blood cells)

The side effects of chemo depend on the type of drugs, the amount taken, and the length of treatment.

Some drugs can have other side effects. For example, drugs such as vincristine or paclitaxel can damage nerves (called neuropathy), sometimes leading to numbness, tingling, or pain, particularly in your fingers and toes. This can also cause some weakness in your arms and legs. These problems tend to be worse in AIDS patients because the AIDS virus affects bone marrow and often nerve cells.

Most side effects go away once treatment is finished, but some can last a long time (or even be permanent). Be sure to ask your doctor about the possible side effects from the chemo drugs that you will receive. There are often ways to prevent or lessen these side effects. For example, drugs can be given to help nausea and vomiting. Tell your medical team about any side effects or changes you notice while getting chemo so that they can be treated promptly.

Biologic therapy (immunotherapy) for Kaposi sarcoma

Biologic therapy, also known as immunotherapy, uses chemicals made naturally by the body (or man-made forms of these chemicals) to help the immune system attack cancer cells.

One of the first drugs used to treat Kaposi sarcoma (KS), interferon alfa, is an example of biologic therapy. For KS, interferon is injected daily into a muscle (called IM) or under the skin (called sub-q). Interferon seems to work by preventing viruses from reproducing and by activating immune system cells that attack and destroy the virus.

About half of patients with good immune function improve when given high doses of these drugs, but patients with fevers, infections, weight loss, or low CD4 counts rarely respond to interferon. Even when treatment does work, it can take several months or more to see a response.

The most common side effects of interferon therapy are flu-like symptoms (fever, pain, and weakness). Treatment with interferon can also cause low blood counts, liver problems, and confusion.

Interferon alfa is not often used now because of its side effects and because it doesn’t work well in many patients with AIDS. Other forms of immunotherapy are now being studied for use in treating KS.

Complementary and alternative therapies for Kaposi sarcoma

When you have cancer you are likely to hear about ways to treat your cancer or relieve symptoms that your doctor hasn’t mentioned. Everyone from friends and family to Internet groups and Web sites may offer ideas for what might help you. These methods can include vitamins, herbs, and special diets, or other methods such as acupuncture or massage, to name a few.

What exactly are complementary and alternative therapies?

Not everyone uses these terms the same way, and they refer to many different methods, so it can be confusing. We use complementary to refer to treatments that are used along with your regular medical care. Alternative treatments are used instead of a doctor’s medical treatment.

Complementary methods: Most complementary treatment methods are not offered as cures for cancer. Mainly, they are used to help you feel better. Some methods that are used along with regular treatment are meditation to reduce stress, acupuncture to help relieve pain, or peppermint tea to relieve nausea. Some complementary methods are known to help, while others have not been tested. Some have been proven not be helpful, and a few have even been found harmful.

Alternative treatments: Alternative treatments may be offered as cancer cures. These treatments have not been proven safe and effective in clinical trials. Some of these methods may pose danger, or have life-threatening side effects. But the biggest danger in most cases is that you may lose the chance to be helped by standard medical treatment. Delays or interruptions in your medical treatments may give the cancer more time to grow and make it less likely that treatment will help.

General considerations in the treatment of Kaposi sarcoma

Different treatment options for Kaposi sarcoma (KS) were discussed in the previous sections. Deciding which treatment to use depends on a number of factors, such as

The type of KS (which helps predict how fast the disease may grow and spread)

The number and location of the KS lesions

What kinds of problems the KS is causing

The person’s overall health

These factors need to be considered because certain treatments, such as chemotherapy, can have serious side effects. Someone who is weak or sick from other problems may not be able to tolerate chemotherapy. In a case like this, the chemo may do more harm than good.

AIDS-related Kaposi sarcoma: For someone with AIDS, the most important part of KS treatment is treating the HIV infection with modern anti-AIDS drug combinations. In many patients, KS lesions begin to get smaller as their immune function gets better. In some patients with AIDS, highly active antiretroviral therapy (HAART) may be the only treatment needed to treat the KS. HAART also helps AIDS patients live longer and feel better. Still, other treatments for KS may be needed to improve symptoms (like pain and swelling).

A local treatment such as radiation therapy, cryosurgery, or a topical retinoid, may be used if a person has only a few skin lesions. KS tumors of the skin, mouth, or anus are sometimes treated with low-doses of radiation therapy. As a rule, doctors use radiation therapy to relieve symptoms or treat highly visible lesions. Sometimes radiation is given to patients who can’t have chemotherapy because they are too weak or have poor liver function.

Chemotherapy may be added to HAART for patients with:

Many skin or mouth lesions

Severe swelling from KS (lymphedema)

Lung lesions causing shortness of breath

Lesions in the stomach and intestines that have caused anemia (low red blood cell count), weight loss, or other problems

For chemotherapy, paclitaxel or one of the liposomal anthracyclines is usually given. If those drugs do not work, other chemotherapy drugs can be tried .

Classic Kaposi sarcoma

Classic KS grows and spreads slowly, so lesions are more often treated with surgery or one of the local treatments, such as radiation therapy.

Chemotherapy may be used for widespread skin lesions or for KS that is in the lymph nodes, the lungs, or the digestive tract. Liposomal anthracyclines or paclitaxel are the drugs most often used for chemotherapy.

Transplant-related Kaposi sarcoma

In people who have had organ transplants, KS lesions sometimes go away on their own if the drugs that suppress the immune system are changed or stopped. A drug called sirolimus may be used in place of another anti-rejection drug because it can often make KS lesions get smaller.

Skin lesions can be treated with radiation therapy or another local treatment. Most doctors try to avoid giving chemotherapy in KS patients who have had organ transplants. But some patients may agree to take part in clinical trials of new drugs.

Endemic Kaposi sarcoma

Because endemic KS occurs in poor countries, treatment options are often limited. When available, the same treatments given for classic KS may be used.

Kaposi sarcoma in HIV-negative men having sex with men

This form of the disease is similar to classic KS, but it occurs in younger men. It is treated like classic KS.

What happens after treatment for Kaposi sarcoma?

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.

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. 

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.

If treatment for Kaposi sarcoma stops working

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. 

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.

What’s new in Kaposi sarcoma research and treatment?

A great deal of research is being done to find more effective ways to prevent and treat Kaposi sarcoma (KS).

Prevention

Probably the most important advance in the prevention of AIDS-related KS has been the development of drugs that help control HIV infection and AIDS. This has reduced the chance of getting KS.

Testing for Kaposi sarcoma herpesvirus (KSHV), the virus that causes KS, could help manage patients at risk for KS, including those infected with HIV or those who will be having an organ transplant and will be taking drugs to suppress their immune system.

Several drugs used to treat related herpesviruses such as cytomegalovirus (CMV) can also help treat KSHV infections. These drugs stop the KSHV-infected cells from making more of the virus. The drugs seem to lower the risk of developing of KS in patients who are at high risk, although they haven’t been found to help treat KS once it has developed.

Treatment

Researchers are studying new and different ways to treat KS.

KS lesions depend on the formation of new blood vessels for their growth. Drugs called angiogenesis inhibitors, which block the growth of blood vessels within tumors, may help treat these lesions. For example, the drug bevacizumab(Avastin) has been shown to cause some KS lesions to shrink or stop growing in a small study. Further studies of this drug and other angiogenesis inhibitors are now being done.

Boosting the immune system is another promising approach to treating KS. Interferon alfa was used for many years to help treat KS, although its use is limited today because of its side effects. Studies looking at similar drugs, such a sinterleukin-12 (IL-12), have shown good results in early studies.

Drugs called immunomodulating agents both boost the immune system and affect blood vessel growth, so these drugs may be helpful against KS. The oldest of these drugs, thalidomide (Thalomid), has been shown to help shrink some KS lesions in early studies, but this drug can have side effects that make it hard to take. Related drugs, such as lenalidomide (Revlimid) and pomalidomide (Pomalyst), which tend to have fewer side effects, are now being studied.

Many other new drugs are also being studied for use against KS, including bortezomib (Velcade) and sorafenib(Nexavar).

Of course, research into HIV vaccines and antiretroviral drugs also may have a great impact on AIDS-related KS. KSHV also offers a new target for KS drugs and biologic therapy. Clinical trials are testing whether antiviral drugs that target KSHV may be used for KS.

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