The malignant potential of HIV-associated Kaposi sarcoma.

The malignant potential of HIV-associated Kaposi sarcoma.

Cancer Cell Int. 2008

Wood NH, Feller L.

ABSTRACT: Human herpesvirus (HHV)-8 associated oncogenesis, a state of immune impairment, a local inflammatory environment, angiogenesis and HIV infection occurring concurrently are important factors for the development of HIV-associated Kaposi sarcoma (KS). Activation of the interleukin (IL)-6 receptor signalling pathway and constitutive signalling of viral G protein-coupled receptor (vGPCR) play an important role in the activation, proliferation and transformation of HHV-8 infected endothelial cells thus contributing to the initiation and progression of KS. HIV-tat protein, HIV-induced immune suppression and a hyperinflammatory state facilitate the oncogenic activity of HHV-8. In this article we reviewed some aspects of HIV-KS pathogenesis and tried to establish, according to the available information in the literature, whether HIV-KS is a monoclonal neoplasm or a benign angioproliferative disorder. From the data of this review it is evident that most of the HIV-KS lesions are oligoclonal in origin. It remains to be demonstrated whether these multiple monoclonal populations of cells are neoplastic, harbouring specific cytogenetic alterations such as mutations, rearrangements and amplifications, or are, as the current evidence shows, the result of HHV-8 induced intracellular signalling pathways that modulate the expression of cellular genes associated with cell cycle regulation, apoptosis, inflammatory response and angiogenesis, and represent a reactive angioproliferative disorder.

Cancer Cell International

Relationship between oral Kaposi 's sarcoma and HAART: contribution of two case reports.

Relationship between oral Kaposi 's sarcoma and HAART: contribution of two case reports.

Med Oral Patol Oral Cir Bucal. 2008 Nov

Campo-Trapero J, Del Romero-Guerrero J, Cano-Sánchez J, Rodríguez-Martín C, Martínez-González JM, Bascones-Martínez A.
Departamento de Medicina y Cirugía Bucofacial, Facultad de Odontología, UCM. Avda Complutense s/n, 28080 Madrid, Spain. jcampo@odon.ucm.es

Two HIV infected patients not receiving Highly Active Antiretroviral Treatment (HAART) presented with epidemic Kaposi's sarcoma of the oral cavity. One patient initially refused HAART, but when the lesion became large enough to be noticeable he agreed to HAART associated with excision of the intraoral lesion by CO2 laser. The other patient developed KS and progressed to AIDS at two years after ceasing HAART due to adverse effects; he was referred to hospital for renewed administration of HAART. In both cases, the lesions observed in the oral cavity were the first clinical manifestation of AIDS. These reports underline the close relationship between the use of HAART and the control of KS lesions, highlighting the important role of the dentist in the identification and early diagnosis of these oral lesions.

Oral Medicine

Imaging techniques for Kaposi's sarcoma.

Imaging techniques for Kaposi's sarcoma.
J HIV Ther. 2008 Sept

O'Mahony D, Gandjbakche A, Hassan M, Vogel A, Yarchoan R.
HIV and AIDS Malignancy Branch, National Cancer Institute, Bethesda, MD, USA.

Kaposi[']s sarcoma (KS) is a multicentric tumour that most frequently involves the skin but can involve other tissues as well. Clinicians treating patients with KS or conducting clinical trials in this disease can benefit from imaging studies to document the extent of disease, to document changes with therapy, and to assess the extent of visceral and lymphatic involvement. A number of conventional techniques can be of use in meeting these needs, such as conventional light photography to assess skin or mucosal lesions, computed tomography of the chest to assess pulmonary disease, and magnetic resonance imaging. In addition, a number of techniques are being developed with the goals of providing improved differentiation of KS from other diseases or providing information about the degree of angiogenesis in the lesions and other physiological factors. We present here an overview of both established and experimental modalities of imaging in KS.

PMID: 19039297 [PubMed - in process]

Imiquimod 5% cream for treatment of HIV-negative Kaposi's sarcoma skin lesions: A phase I to II, open-label trial in 17 patients.

Imiquimod 5% cream for treatment of HIV-negative Kaposi's sarcoma skin lesions: A phase I to II, open-label trial in 17 patients.
J Am Acad Dermatol. 2008 Apr
Célestin Schartz NE, Chevret S, Paz C, Kerob D, Verola O, Morel P, Lebbé C.
Department of Dermatology 2, Hôpital Saint Louis AP-HP, Paris, France. noel.schartz@noos.fr

BACKGROUND: Kaposi's sarcoma (KS), a virus-associated neoplasm, can be treated locally or systemically with interferon alfa. Therefore, imiquimod, an immune response modifier able to induce interferon-alpha secretion in situ, could prove a good local treatment for KS skin lesions.

OBJECTIVE: We sought to determine the efficacy and safety of imiquimod 5% cream for the topical treatment of classic or endemic KS skin lesions in patients who are HIV negative.

METHODS: We conducted a prospective, open-label, single center, phase II clinical trial. Imiquimod cream was applied under occlusion 3 times a week for 24 weeks. The main efficacy end points were the safety of topical imiquimod and the overall clinical response in patients evaluated on the basis of modified AIDS Clinical Trials Group criteria at 36 weeks. The statistical analysis was based on the intent-to-treat data set.

RESULTS: Seventeen patients were enrolled. Eight (47%) presented objective overall clinical response (2 complete and 6 partial responses). Tumor progression was noted in 6 patients. The most frequent side effects were local itching and erythema, seen in 9 patients (53%).

LIMITATIONS: This was not a randomized placebo-controlled study and was restricted to a small number of patients.

CONCLUSION: Topical imiquimod 5% cream had antitumor activity in about half the patients with classic and endemic KS and was generally well tolerated.

Journal of American Academy of Dermatology

Classic Kaposi Sarcoma in the United States over the last two decades:

Classic Kaposi Sarcoma in the United States over the last two decades: a clinicopathologic and molecular study of 438 non-HIV-related Kaposi Sarcoma patients with comparison to HIV-related Kaposi Sarcoma.
Mod Pathol. 2008
Hiatt KM, Nelson AM, Lichy JH, Fanburg-Smith JC.
1Dermatopathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

Classic Kaposi sarcoma is rare and occurs predominantly in Mediterranean and Middle Eastern men. Since the emergence of acquired immune deficiency syndrome (AIDS)-related Kaposi sarcoma, the incidence, clinicopathologic features, and molecular human herpesvirus 8 (HHV-8) association of American Classic Kaposi Sarcoma has not been fully explored. This study compares Classic Kaposi Sarcoma to AIDS-related Kaposi Sarcoma over the same two decade time period. There were 438 histologically and clinically confirmed Classic Kaposi Sarcoma patients. The ethnic/racial distribution included Caucasian/American (56%), Mediterranean (22%), South American Hispanic (18%), Black (10%), western European (4%), Middle East (4%), Scandinavian (2%), and other (2%). Classic Kaposi Sarcoma was more common in men, 7:1, with a mean age of 74 years. The lesions presented in the lower extremity (69%), in the nodular stage (83%), and HHV-8 was detected by PCR in 40/41 randomly selected cases. A second, non-Classic Kaposi Sarcoma, malignancy was present in 42% (n=45) of the 108 Classic Kaposi Sarcoma patients with complete clinical information, 73% (33 patients) with a higher incidence over the general population. Follow-up of <1-19> revealed that 24% of patients died of second malignancy, 22% died of other medical conditions, 2% died of treatment-related complications, and 2% patients died of widespread disease. Thirty-five percent are alive with no evidence of disease and 15% with persistent disease. Human immunodeficiency virus-related Kaposi Sarcoma was observed in 354 cases. There was a male predominance and more aggressive behavior, with higher rates of visceral and disseminated disease. While Classic Kaposi Sarcoma in the United States is an indolent disease and rarely accounts for patient demise, predominantly affecting Caucasian/American males on the lower extremity in the nodular phase, it more importantly may denote an underlying other malignancy. Current PCR probes detect HHV-8 in 98% of Classic Kaposi Sarcoma cases. In comparison, AIDS-related Kaposi Sarcoma is predominately multicentric, visceral, and disseminated, with more aggressive behavior.

PubMed

Linking the Kaposi's Sarcoma-Associated Herpesvirus (KSHV/HHV-8) to Human Malignancies.

Linking the Kaposi's Sarcoma-Associated Herpesvirus (KSHV/HHV-8) to Human Malignancies.

Methods Mol Biol. 2009
Kalt I, Masa SR, Sarid R.
The Mina and Everard Goodman Faculty of Life Sciences, Bar-Ilan University, Ramat Gan, Israel.

In 1994, the Kaposi's sarcoma-associated herpesvirus (KSHV/HHV-8) was identified as the etiologic agent of Kaposi's sarcoma (KS). KSHV has since been associated with two additional malignancies: primary effusion lymphoma and multicentric Castleman's disease. In this chapter, we describe the current understanding of the pathogenesis, transmission, and prevalence of KSHV, and its association mainly with KS. We describe evidence demonstrating that KSHV is a causative agent for KS, and we present other factors that possibly contribute to the incidence of KS. We compare worldwide data on the prevalence of KS and of KSHV infection. Specific viral genes that may induce KS tumors or enable their growth also are described. Finally, we discuss the implications of the transmission modes and epidemiology of this virus on recommendations for KSHV screening of tissues and blood products before transplantation or transfusion.

Springer Link

Kaposi's sarcoma following living donor kidney transplantation: review of 7,939 recipients

Kaposi's sarcoma following living donor kidney transplantation: review of 7,939 recipients

Int Urol Nephrol. 2008 Nov 14

Einollahi B, Lessan-Pezeshki M, Nourbala MH, Simforoosh N, Pourfarziani V, Nemati E, Nafar M, Basiri A, Pour-Reza-Gholi F, Firoozan A, Ghadiani MH, Makhdoomi K, Ghafari A, Ahmadpour P, Oliaei F, Ardalan MR, Makhlogh A, Samimagham HR, Azmandian J.
Nephrology Research Center, Baqiyatallah University of Medical Sciences, Mollasadra St. Vanak Sq., Tehran, Iran, behzad.einollahi@gmail.com.

INTRODUCTION: Kaposi's sarcoma (KS) is one of the most common tumors to occur in kidney recipients, especially in the Middle East countries. Limited data with adequate sample size exist about the development of KS in living kidney recipients.

METHODS: Therefore, we made a plan for a multicenter study, accounting for up to 36% (n = 7,939) of all kidney transplantation in Iran, to determine the incidence of KS after kidney transplantation between 1984 and 2007.

RESULTS: Fifty-five (0.69%) recipients who developed KS after kidney transplantation were retrospectively evaluated with a median follow-up of 24 (1-180) months. KS occurred more often in older age when compared to patients without KS (49 +/- 12 vs. 38 +/- 15 years, P = 0.000). KS was frequently found during the first 2 years after transplantation (72.7%). Skin involvement was universal. Furthermore, overall mortality rate was 18%, and it was higher in patients with visceral involvement compared to those with mucocutaneous lesions (P = 0.01). However, KS had no adverse affect on patient and graft survival rates compared to those without KS. Forty-four patients with limited mucocutaneous disease and four with visceral disease responded to withdrawal or reduction of immunosuppression with or without other treatment modalities. Renal function was preserved when immunosuppression was reduced instead of withdrawn in patients with and without visceral involvement (P = 0.001 and 0.008, respectively).

CONCLUSION: The high incidence of KS in this large population studied, as compared to that reported in other transplant patient groups, suggests that genetic predisposition may play a pathogenetic role.

SpringerLink

CD8+ T cell immunity to Epstein-Barr virus and Kaposi's sarcoma-associated herpesvirus.

CD8+ T cell immunity to Epstein-Barr virus and Kaposi's sarcoma-associated herpesvirus.

Semin Cancer Biol. 2008 Nov 1

Hislop AD, Sabbah S.
CRUK Institute for Cancer Studies, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; MRC Centre for Immune Regulation, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom.

Gammaherpesviruses are agents which have evolved to persist within the lymphoid system and many have oncogenic potential; studying gammaherpesvirus infections therefore has the potential to reveal much about the workings of the immune system and the control over viral oncogenesis. The lymphocryptovirus Epstein-Barr virus (EBV) and the rhadinovirus Kaposi's sarcoma-associated herpesvirus (KSHV, also known as human herpesvirus 8) are the two human gammaherpesviruses. Analysis of the T cell response to EBV has guided understanding of immunity to infection and disease caused by this virus, as well as directed the development of vaccination and therapeutic interventions in EBV-associated disease. Less is known about the T cell response to KSHV and its exact role in controlling virus infection and disease. Here we discuss the CD8+ T cell response to these two gammaherpesviruses.

ScienceDirect

Relationship between oral Kaposi 's sarcoma and HAART: contribution of two case reports.

Relationship between oral Kaposi 's sarcoma and HAART: contribution of two case reports.
Med Oral Patol Oral Cir Bucal. 2008 Nov

Campo-Trapero J, Del Romero-Guerrero J, Cano-Sánchez J, Rodríguez-Martín C, Martínez-González JM, Bascones-Martínez A.

Departamento de Medicina y Cirugía Bucofacial, Facultad de Odontología, UCM. Avda Complutense s/n, 28080 Madrid, Spain. jcampo@odon.ucm.es

Two HIV infected patients not receiving Highly Active Antiretroviral Treatment (HAART) presented with epidemic Kaposi's sarcoma of the oral cavity. One patient initially refused HAART, but when the lesion became large enough to be noticeable he agreed to HAART associated with excision of the intraoral lesion by CO2 laser. The other patient developed KS and progressed to AIDS at two years after ceasing HAART due to adverse effects; he was referred to hospital for renewed administration of HAART. In both cases, the lesions observed in the oral cavity were the first clinical manifestation of AIDS. These reports underline the close relationship between the use of HAART and the control of KS lesions, highlighting the important role of the dentist in the identification and early diagnosis of these oral lesions.

Full Text Article

Kaposi’s Sarcoma, Leg or Arm Swelling and Lymphedema

Kaposi’s Sarcoma and Lymphedema

A Review of the Literature When Kaposi’s Sarcoma burst into the news some 20 years ago, it quickly became known as a cancer associated with HIV/AIDS. Interestingly, KS has shown that in and of itself, the cancer causes lymphedema by damaging the lymphatic system. Since that time, however, there have been two more groups of patients who have been clinically shown to be at risk. The first groups is that of organ transplants. Indeed, a study done by the Mayo Clinic in Rochester, Minnesota demonstrated that the risk of contracting Kaposi’s Sarcoma is 500 times greater in the organ transplant population then that of the general population. The reason for this is simple. Organ transplant patients must take immunosuppressive drugs to prevent organ rejection. The next group that is now showing a proclivity towards Kaposi’s Sarcoma is comprised of long term lymphedema patients. This group includes both primary and secondary lymphedema. A lymphedematous limb has long been understood to become immunodeficient and there is even conjecture from early studies that indeed even those with secondary lymphedema may already have an “at risk” lymph system, thereby possibly causing a lowered immune response throughout their body.

Arm and Leg Swelling After Cancer

With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically. With this progress, a new and often misunderstood and misdiagnosed complication has arisen.

Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.

This swelling occurs because of one of several factors.

First, the swelling begins after lymph nodes have been removed for cancer biopsies.

Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.

Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids. The fluids collect in the limbs effected and swelling beings.

This swelling is called lymphedema. The swelling that occurs is permanent, and while it is not curable it is treatable.

Permanent Leg Swelling

****In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered****

There are several groups of people who experience leg swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.

Group One

This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.

Group Two

Another extremely large group that experiences permanent leg swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema. What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid. In cancer patients this is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries. This is also referred to as secondary lymphedema.

Group Three

Group three consists of people who have leg swelling from seemingly unknown reasons. There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.

The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later. This type of leg swelling is called primary lymphedema. It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system. This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications. Treatment should NOT include the use of diuretics.

What is Lymphedema?

Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg. The accumulation of fluid causes the permanent swelling caused by a defective lymph system.

A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.

What are the symptoms of Lymphedema?

If you are an at risk person for leg lymphedema there are early warning signs you should be aware of. If you experience any or several of these symptoms, you should immediately make your physician aware of them.

1.) Unexplained aching, hurting or pain in the leg.

2.) Experiencing "fleeting lymphedema." This is where the limb may swell, even slightly, then return to normal. This may be a precursor to full blown leg lymphedema.

3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the foot, or between the ankle and knee. This is an indication of early lymphatic malfunction.

4.) Any arm inflammation, redness or infection.

5.) You may experience a feeling of tightness, heaviness or weakness of the leg.

How is Lymphedema Treated?

The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MDT), there are variances, but most involve these two type of treatment. It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally. With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.

What are some of the complications of lymphedema?

1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immuno-deficient.

2. Draining wounds that leak lymphorrea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.

3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.

4. Loss of Function due to the swelling and limb changes.

5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.

6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.

7. Sepsis, Gangrene are possibilities as a result of the infections.

8. Possible amputation of the limb.

9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.

10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.

11. Chronic localized inflammations.

Can lymphedema be cured?

No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided. Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.

What is Kaposi's sarcoma?

What is Kaposi's sarcoma?

Although Kaposi's sarcoma (KS) is a type of cancer, it differs from other types of cancer in the way it develops. Unlike most cancers, which start in one place and may then spread to other parts of the body, KS can appear in several parts of the body at the same time. The most common site for KS is on the skin but it may also affect internal organs, particularly the lymph nodes (part of your immune system), the lungs and parts of the digestive system (the gut).

Causes of Kaposi's sarcoma

Most KS is now believed to be caused by a virus called Human Herpes Virus 8 (HHV8), which is also known as Kaposi’s Sarcoma-associated Herpes Virus (KSHV). It can affect people with a weakened immune system, including people with HIV (Human Immunodeficiency Virus) and Aids.

HHV8 is mainly spread through saliva, but can also be spread in blood and semen, or from a mother to her unborn child. The virus can be passed on through sexual contact, kissing, blood transfusions and organ transplantation.

Types of Kaposi’s Sarcoma

There are four main types of KS They are all more common in men than women.

I. Classic Kaposi’s Sarcoma

The first, called classic KS, develops without any weakened immunity, like many other types of cancer. It is very uncommon and is usually only found in older men of Mediterranean, Middle Eastern, or Jewish descent.

This type of KS is usually only found in the skin, particularly on the lower legs and feet.

As it is a slow-growing cancer, people with early stage classic KS may not need any treatment.

II. Endemic or African Kaposi’s Sarcoma

The second type of KS, endemic or African KS, is found in parts of sub-Saharan Africa. It develops more quickly than classic KS and can affect men, women and children of all ages, although it is more common in men.

III. Aids-related Kaposi’s Sarcoma

The third type of KS, Aids-related KS, is the commonest of the four types, although it has become less common as the treatment of HIV and Aids has improved. As HIV infection develops, the immune system becomes weaker and the risk of developing KS increases.

IV. Transplant-related Kaposi’s Sarcoma

The fourth type of KS usually occurs in people who have a weakened or damaged immune system. People who have had organ transplants, such as a kidney transplant or a bone marrow transplant from a donor, take medicines which suppress their immune system. This is to reduce the risk of their body rejecting the donated organ. Although rare, KS can occur in transplant patients who are taking these immunosuppressant drugs. This type of KS may improve if the immunosuppressant drugs are reduced or stopped.

Signs and Symptoms of Kaposi’s Sarcoma

Kaposi's sarcoma on the skin appears as a small, painless, flat area (lesion) or lump, ranging in colour from brown or brown-red to reddish purple. The lesions or lumps can develop quickly. Although there may be a single area at first, it is not uncommon for more than one to appear.

Any part of the skin, including the inside of the mouth, can be affected. Often the lumps merge to form a larger tumour.

KS can also affect other parts of the body, most commonly the lymph nodes, the lungs and the organs of the digestive system. The signs and symptoms of internal KS depend on the area of the body that is affected. If the lymph nodes are affected, which is common in Aids-related KS, there may be swelling in the limbs. This is known as lymphoedema and is caused by the KS cells blocking the lymph nodes and disrupting the normal circulation of lymph fluid around the body.

Lymphoedema can be a distressing and uncomfortable symptom, and while there is no actual 'cure' there are ways to relieve it.

Our section on lymphoedema gives useful advice about reducing lymphoedema through methods such as exercise and massage.

KS in the lungs commonly causes breathlessness, while a tumour in the digestive system (gut) may cause nausea, vomiting, and bleeding.

How Kaposi’s Sarcoma is diagnosed

You may begin by seeing your GP, who will examine you and check your general health. Your GP will arrange for you to have some tests. You may be referred to a hospital for specialist advice and treatment.

If you already know you have HIV or Aids, you will probably begin by seeing your HIV specialist.

At the hospital you will need to have blood tests and a full examination. Your whole body will be checked, as the lesions can be found on any areas of the skin, including the inside of the mouth, the palms of the hands, the scalp, and the soles of the feet. Although your doctor may suspect that you have KS by simply looking at the lesions, a biopsy is usually done to confirm the diagnosis.

Biopsy

This is generally a quick and simple procedure, which can often be done in an outpatient department. Using a local anaesthetic to numb the area, the doctor removes a small piece of the lesion or lump for examination under a microscope. The area may be sore for a few days afterwards. Further Tests If a diagnosis of KS is confirmed, further tests are usually done to see if there are any signs of KS cells elsewhere in your body.

These may include any of the following:

Chest x-ray

This may be done to check for signs of KS in your lungs and airways (bronchial passages). As lung infections are common in people with Aids, it may be difficult in this situation to tell whether changes on the x-ray are actually caused by KS. A CT scan may be done to confirm the diagnosis.

CT (computerised tomography) Scan

A CT scan takes a series of x-rays which builds up a three-dimensional picture of the inside of the body. The scan is painless but takes from 10–30 minutes. It can help to show whether there are any signs of KS in other parts of the body. It is particularly useful for diagnosing KS in the lymph nodes.

CT scans use a small amount of radiation, which will be very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan. You may be given a drink or injection of a dye which allows particular areas to be seen more clearly. For a few minutes, this may make you feel hot all over. If you are allergic to iodine or have asthma you could have a more serious reaction to the injection, so it is important to let your doctor know beforehand.

You will probably be able to go home as soon as the scan is over.

Endoscopy

This procedure enables the doctor to look inside the body, through a thin flexible tube called an endoscope that is passed down your throat. The endoscope has a tiny camera and a light on the end. If necessary, the doctor can take a small sample of the cells (a biopsy) to be examined under a microscope.

A mild sedative helps you to relax during the test and a local anaesthetic will be sprayed onto your throat to prevent any discomfort as the tube is passed down. The doctor or nurse endoscopist can examine your windpipe, lungs (bronchoscopy), oesophagus and stomach (gastroscopy) in this way, and check for any signs of KS.

Colonscopy

Another type of endoscopy is used to examine your large bowel (colon). This test is called a colonoscopy. The bowel must be empty for this test so a careful diet must be followed for a few days beforehand and you may be given laxatives to take. A bowel wash-out is usually done just before the test. This involves a nurse gently passing a tube into your back passage while you are lying on your left side. Water is then passed through the tube. You will be asked to hold the liquid in your bowel for a few minutes before you go to the toilet.

Just before the colonoscopy you may be given a mild sedative to help you to relax. Once you are lying comfortably on your side the doctor or specialist nurse will gently pass a flexible tube (called a colonoscope) into your back passage. The tube can easily pass around the curves of the bowel, and the lining of most of the bowel can be examined. A light and camera on the inside of the tube helps the doctor or nurse to see any abnormal areas or swelling.

Most people are ready to go home a couple of hours after their test. It is a good idea to arrange for someone to collect you from the hospital, as it is best not to drive for several hours after a sedative.

Lung Function test

If tests show that your lungs have been affected you may have lung function tests to check how well your lungs are working. You will be asked to blow into a machine so that a series of measurements can be taken to show how well your lungs are working.

Treatment for Kaposi’s Sarcoma

Types of Treatment

Mild cases of Kaposi's sarcoma may not need to be treated. It may be possible to use skin-camouflage to cover any skin lesions. However, if the lesions or lumps are causing embarrassment and distress, treatment may be recommended.

Your doctor is more likely to suggest that you have treatment if your KS is affecting internal organs, or if there are many skin lesions. If you have HIV that is not very well controlled, your specialist may also recommend that you start anti-HIV treatment. The type of treatment used will depend on a number of different factors, including the size and position of the tumours and your general health.

· Classic KS does not usually require any treatment, although radiotherapy is sometimes used for larger or easily visible lesions.

· Endemic or African KS is often treated with chemotherapy.

· Treatment of Aids-related KS is often affected by the person's general health. As the immune system has already been weakened by the illness, extra care has to be taken to ensure that any side effects of treatment are not going to make your health become worse. For most people with Aids-related KS the treatment will include highly active antiretroviral therapy (HAART) which reduces the level of the HIV virus in the body and improves immunity.

· Transplant-related KS, caused by immunosuppressant drugs, can sometimes be controlled by stopping or reducing these drugs.

If you have any questions about your treatment, don't be afraid to ask your doctor or the nurse looking after you. It often helps to make a list of questions for your doctor and to take a close friend or relative with you to your appointment.

How treatment is planned

In most hospitals a team of specialists will decide the treatment that is best for you. This multidisciplinary team (MDT) will often include:

· virologist – a doctor that specialises in treating people with viruses

· oncologists – doctors who have experience treating KS using chemotherapy and radiotherapy · specialist nurses who give information and support

· radiologists who help to analyse x-rays

· an HIV specialist.

The MDT may also include a number of other healthcare professionals, such as a:

· dietitian

· physiotherapist

· occupational therapist

· psychologist or counsellor.

Together they will be able to advise you on the best course of action and plan your treatment taking into account a number of factors. These include your age, general health, and the size and position of the tumours. Remember to ask questions about any aspects that you do not understand or feel worried about. You may find it helpful to discuss the benefits and disadvantages of each option with your doctor, specialist nurse, or with the nurses on our cancer information and support service. Giving your consent .

Before you have any treatment, your doctor will explain the aims of the treatment to you. They will usually ask you to sign a form saying that you give your permission (consent) for the hospital staff to give you the treatment.

No medical treatment can be given without your consent, and before you are asked to sign the form you should have been given full information about:

· the type and extent of the treatment you are advised to have

· the advantages and disadvantages of the treatment

· any possible other treatments that may be available

· any significant risks or side effects of the treatment.

If you do not understand what you have been told, let the staff know straight away so that they can explain again. Some cancer treatments are complex, so it is not unusual for people to need repeated explanations. It is often a good idea to have a friend or relative with you when the treatment is explained, to help you remember the discussion more fully. You may also find it useful to write down a list of questions before you go to your appointment.

People often feel that the hospital staff are too busy to answer their questions, but it is important for you to be aware of how the treatment is likely to affect you. The staff should be willing to make time for you to ask questions.

You can always ask for more time to decide about the treatment if you feel that you can’t make a decision when it is first explained to you.

You are also free to choose not to have the treatment. The staff can explain what may happen if you do not have it. It is important to tell a doctor, or the nurse in charge, so that they can record your decision in your medical notes. You do not have to give a reason for not wanting to have treatment, but it can be helpful to let the staff know your concerns so that they can give you the best advice.

Second opinion

Usually a number of cancer specialists work together as a team and they use national treatment guidelines to decide on the most suitable treatment for a patient. Even so, you may want to have another medical opinion. Either your specialist, or your GP, will be willing to refer you to another specialist for a second opinion, if you feel it will be helpful. The second opinion may cause a delay in the start of your treatment, so you and your doctor need to be confident that it will give you useful information.

If you decide to have a second opinion, it may be a good idea to take a friend or relative with you, and have a list of questions ready, so that you can make sure your concerns are covered during the discussion.

Anti-HIV Drugs

Most people with Aids-related Kaposi's sarcoma will be offered treatment with anti-HIV drugs (antiretroviral therapy). A combination of some of the antiretroviral drugs (usually three or more) will usually be used. To be effective, antiretroviral drugs need to be taken every day, often for the rest of your life. Your doctor will discuss the benefits and possible side effects with you if they are appropriate in your situation.

These drugs can stop the HIV virus from multiplying inside the cells that it has affected, and can stop the virus from infecting other cells. This reduces the amount of HIV in the body and limits the damage that the virus can do to the immune system. In many cases, antiretroviral therapy alone can control KS, although this may take many months.

There are different types of anti-HIV drugs. Commonly used types are

· Nucleoside reverse transcriptase inhibitors (NRTIs) such as abacavir (ABC), didanosine (ddI), emtricitabine lamiduvine (3TC), stavudine (d4T), and zidovuvine (AZT)

· Nucleotide reverse transcriptase inhibitors (NtRTIs) such as tenofovir (TFV)

· Protease inhibitors (PIs) such as amprenavir, atazanavir, indinavir, lopinavir, nelfinavir, ritonavir, and saquinavir

· Non-nucleoside reverse transcriptase inhibitors (NNRTIs) such as efavirenz and nevirapine

· Other antiretrovirals such as enfuvirtide.

You can get more information about anti-HIV treatment from some of the specialist HIV and Aids organisations.

Treating Kaposi’s Sarcoma with surgery

Surgery may be used to remove Kaposi's sarcoma from the skin, particularly when the lesions are small. This is a simple procedure and may be done in the outpatient clinic or day ward. The doctor injects local anaesthetic into the skin around the lesions to numb the area. The lesions are removed and the wound stitched. This will leave a small scar which gradually fades.

Some KS lesions can be removed using cryotherapy, which freezes the area to destroy the cancer cells. Sometimes, a laser may be used to burn away the lesion.

Your doctor or specialist nurse can give you more information

Treating Kaposi Sarcoma with Chemo therapy

This is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. They work by disrupting the growth of cancer cells. Commonly used chemotherapy drugs to treat Kaposi's sarcoma are doxorubicin, vincristine (Oncovin®), bleomycin, etoposide (Etopophos®, Vepesid®) and paclitaxel (Taxol). Chemotherapy for internal KS The chemotherapy drugs are usually given by drip (infusion) into a vein in your arm (intravenously), but some chemotherapy drugs can be taken as tablets (orally). They are then carried around the body by the bloodstream. Chemotherapy for KS is usually given every three weeks but may be given more frequently, depending on the drugs used. Treatment is often given as an outpatient.

Chemotherapy for Skin KS

If the KS is only affecting the skin, it may be possible to use chemotherapy injected directly into the skin lesion. This is known as intralesional chemotherapy and tends to be used for smaller lesions (less than 0.5cm (¼ inch) wide). It can be a useful alternative to radiotherapy for areas or skin types where radiotherapy might cause some darkening of the skin, particularly on the face. Intralesional chemotherapy can also be used for tumours inside the mouth.

For more extensive skin KS, chemotherapy is usually given as a drip into a vein.

Chemotherapy treatment can shrink the areas of Kaposi's sarcoma in the skin and make them lighter in colour. In some people the lesions may almost disappear completely.

Liposomal Chemotherapy

A different form of chemotherapy, called liposomal chemotherapy, is now often used to treat KS. The molecules of the chemotherapy drugs are enclosed (encapsulated) in a fat-based coating known as a liposome. Liposomes are able to travel to the tumour site, where they release the drug. The advantage of this type of chemotherapy is that there are fewer side effects, which means that treatment can be given over a longer period.

The liposomal chemotherapy drugs that are commonly used to treat KS are liposomal daunoroubicin (Daunoxome®) and liposomal doxorubicin (Caelyx®, Myocet®). These drugs are usually given as drips (infusions) every 2–3 weeks.

Our section on chemotherapy discusses the treatment and its side effects in more detail. Information about individual drugs and their particular side effects are also available.

Side Effects

Chemotherapy can cause unpleasant side effects. However, many people have few side effects, and those that occur can often be well controlled with medicine. The main side effects are described here, along with some of the ways they can be reduced.

Lowered resistance to infection The chemotherapy can reduce the production of white blood cells by the bone marrow, making you more prone to infection. This is a common side effect of chemotherapy and usually begins seven days after treatment has been given. The number of white blood cells in your blood usually reaches its lowest point at 10–14 days after chemotherapy. Your blood cells will then increase steadily and will usually have returned to normal before your next course of chemotherapy is due.

Contact your doctor or the hospital straightaway if:

· Your temperature goes above 38ºC (100.5ºF)

· You suddenly feel unwell (even with a normal temperature).

You will have a blood test before having more chemotherapy, to make sure that your cells have recovered. Occasionally it may be necessary to delay your treatment if your blood count is still low.

Anaemia (low number of red blood cells) While having chemotherapy you may become anaemic. This may make you feel tired and breathless.

Bruising or bleeding

The chemotherapy can also reduce the production of platelets, which help the blood to clot. Let your doctor know if you have any unexplained bruising or bleeding.

Nausea and vomiting

Some of the drugs used to treat KS may cause nausea (feeling sick) and vomiting. There are now very effective anti-sickness drugs (anti-emetics) to prevent or reduce nausea and vomiting. Your doctor will prescribe these for you.

Hair loss

Unfortunately, some chemotherapy drugs can make your hair fall out. You can ask your doctor if the drugs you are taking are likely to cause hair loss or other specific side effects.

People who lose their hair often wear wigs, hats or bandannas. Some people are entitled to a free wig from the NHS and your doctor or nurse will be able to give you more details. If your hair falls out, it will grow back within 3–6 months, once your treatment is over.

Sore mouth Some chemotherapy drugs can make your mouth sore and cause mouth ulcers. Regular mouthwashes can help to keep your mouth clean and relieve any soreness. Your nurse will show you how to use these properly. If you don’t feel like eating during treatment, you could try a diet of soft food or replacing some meals with nutritious drinks.

Skin reaction

Intralessional chemotherapy may cause temporary inflammation of the skin.

Tiredness You may feel tired and have a general feeling of weakness. It is important to allow yourself plenty of time to rest.

Although they may be hard to bear at the time, these side effects will gradually disappear once your treatment is over.

Chemotherapy affects people in different ways. Some people find they are able to lead a fairly normal life during their treatment, but many find they become very tired and have to take things much more slowly. Just do as much as you feel like and try not to overdo it. Treating Kaposi’s Sarcoma with

Radiotherapy

Radiotherapy is a treatment which uses high-energy rays to destroy the Kaposi's sarcoma cells while doing as little harm as possible to normal cells. The radiotherapy is aimed at the skin lesions or internal tumours and is painless. Usually between one and five doses are given. After radiotherapy to the skin, small lesions may fade completely but larger and deeper lumps may become smaller and flatter, similar to a mole. Radiotherapy can be very effective in reducing symptoms of internal KS, particularly swelling, pain and bleeding.

Side Effects

Skin care Side effects of radiotherapy depend on the area of the body which is being treated. Radiotherapy to the skin alone usually causes few side effects. Pale skin around the treated area may become red, sore and itchy. People with darker skins may develop a blue or black tinge in the treated area. Advice about skin care varies from one hospital to another. Some will advise you not to wash the skin at all while you are having radiotherapy. Others will advise you to use only tepid water on the area and to pat it dry with a soft towel. Perfumed creams and lotions should be avoided and you should check with the radiotherapy staff before applying anything to your skin.

If necessary, your doctor will prescribe a special cream to soothe the sore area. Avoid exposing the treated area to the sun or extremes of hot or cold.

Hair loss

Radiotherapy may make your hair fall out in the treated area. Hair in other areas of the body is not affected. Your hair may grow back once your treatment is over, but in some cases the hair loss may be permanent.

Feeling sick Radiotherapy to tumours in or near the digestive system can lead to nausea and vomiting. Anti-sickness (anti-emetic) drugs can often help to overcome this problem. It can also help if you avoid large meals and eat small amounts more often, or supplement your diet with nutritious drinks, which you can buy at most chemists.

Hoarse voice Radiotherapy near the voice box (larynx) may cause hoarseness of the voice. This is usually temporary and should begin to reduce once the treatment has finished.

Tiredness You may feel very tired. It is important to get as much rest as possible, especially if you are travelling a long way for treatment every day.

Most side effects of radiotherapy disappear gradually once your course of treatment is over, although some may continue for several months afterwards. If you have any problems during your treatment, talk to the radiotherapy staff at the hospital, as they have experience of looking after other people in the same situation as yourself.

Radiotherapy does not make you radioactive and it is perfectly safe for you to be with other people, including children, throughout your treatment.

Treating Kaposi's sarcoma with immunotherapy

This type of treatment is occasionally used to treat Kaposi's sarcoma. It is often used alongside other treatments such as anti-HIV therapies. Immunotherapy involves the use of proteins normally produced by the body during viral infections such as flu. These anti-viral proteins can also be produced in a laboratory.

Interferon

Interferon is the most common type of immunotherapy used to treat KS and is usually given three times a week by injection under the skin. Alternatively, it may be injected into the lesion. The needle is very small and fine so the injections are only slightly uncomfortable. You will be taught how to give yourself these injections so you can do them at home.

In the first week or two of treatment, interferon can cause side effects similar to those of flu: especially chills, fever, headaches, tiredness and aching in the back, joints and muscles. However, these soon disappear. Your doctor may recommend that you take paracetamol about half an hour before your injection to prevent these side effects.

Living with Kaposi's sarcoma

Many people with slow-growing or early KS often find that physically they feel quite well, but emotionally may find it difficult to cope. For some people the appearance of KS is the first sign that they have Aids. In this situation, coming to terms with the diagnosis, whether they knew about it beforehand or not, together with the constant physical reminder of their illness, can be devastating.

It often helps to talk to someone who understands the special needs and problems of people with Aids, their partners and families. A large number of organisations offer help and support to people with Aids. Some of the main Aids organisations in the UK are listed in this section (see organisations). Local support groups can also provide support and you can get details of these from The Terrence Higgins Trust.

People affected by Kaposi's sarcoma in the skin may find this distressing. However, there is a way to reduce the differences in skin colour and make the areas of KS less noticeable. Camouflage make-up consists of specially designed creams, and the ranges available are suitable for all skin types and colours, in both men and women. Some clinical nurse specialists, the British Association of Skin Camouflage and the British Red Cross offer a camouflage make-up service with individual teaching sessions on how to apply it for the best effect.

If KS has caused swelling of the limbs and lymph nodes (lymphoedema), you may have hot, overstretched and painful skin in those areas. We have a section on lymphoedema, and our cancer information and support service can give you details of your nearest lymphoedema clinics.

If you are finding it difficult to eat and are losing weight, it may be advisable to take small, frequent, high-calorie, high-protein drinks which are available from chemists and hospital dietitians. If cleaning your teeth is painful because of mouth lesions, try using a soft toothbrush or foam stick.

CancerBacUp