Kaposi’s sarcoma

Kaposi's sarcoma (KS) is a type of soft tissue sarcoma. It usually affects the:

• skin
• lining of the mouth

It rarely affects organs inside the body, like the:

• lungs
• liver
• stomach
• bowel
• lymph nodes

Kaposi's sarcoma can also develop in several parts of the body at the same time.

Kaposi's sarcoma comes in 4 main types. It's more common in men than in women, and it's caused by a virus called human herpes virus 8 (HHV8).

HIV is a virus that attacks the immune system. Your immune system helps protect you from infection and disease.

People who have HIV may have a damaged immune system. If they also have human herpes virus 8 (HHV8), their risk of developing Kaposi's sarcoma goes up.

HIV-related Kaposi's sarcoma (KS) is the most common type of KS in the UK. But it's now much less common, because anti-retroviral drugs help keep HIV under control.

Classic Kaposi's sarcoma is rare. It's more common in older men in Mediterranean or Middle Eastern countries, where the human herpes virus (HHV8) is more common.

This type of Kaposi's sarcoma is normally found only in the skin, particularly on the lower legs and feet. It's a slow-growing cancer.

Endemic Kaposi's sarcoma is found in parts of Africa, where HHV8 is more common. It usually develops in younger people, under the age of 40.

Transplant-related Kaposi's sarcoma can develop in people who have had an organ transplant. The reason is the medicine they have to take. These drugs, called immunosuppressants, hold back the immune system so the body is less likely to reject the donated organ.

You may also hear this type called iatrogenic Kaposi's sarcoma or acquired Kaposi's sarcoma.

The first sign of Kaposi's sarcoma is usually a coloured spot on the skin (a skin lesion). These lesions can vary in colour. They may be pink, brown, brown-red or reddish purple.

A lesion can look like one of these:

• a flat area on the skin, called a patch
• a slightly raised area, called a plaque
• a raised bump, called a nodule

Lesions can develop anywhere on the skin, including inside the mouth. They're more common on the legs, face and groin area.

They may develop quickly. There may be just one lesion at first, but more than one can appear. Lesions can also join together and form a bigger tumour.

You may have general symptoms with Kaposi's sarcoma, like:

• a fever
• weight loss
• tiredness

Other symptoms depend on where in the body the sarcoma is.

If the lymph nodes are affected, they may become swollen. If the lymph vessels are damaged, fluid can build up in the arms, legs, face and genitals. This is called lymphoedema.

If the sarcoma is in the lungs, you may have breathlessness and a cough. If it's in the stomach and bowel, you may have diarrhoea and abdominal pain.

Sometimes the lesions bleed slowly. Over time this may lead to low numbers of red blood cells (anaemia), which can cause symptoms like tiredness and breathlessness.

Kaposi's sarcoma is caused by a virus called human herpes virus 8 (HHV8). You may also see it called Kaposi's sarcoma-associated herpes virus (KSHV).

Most people who carry this virus never get Kaposi's sarcoma, because their immune system keeps the virus under control. The virus is more likely to cause the condition in people whose immune system is weakened or damaged.

The immune system is made up of tissues and organs that work together to protect us against infections. It can be weakened or damaged in different ways, for example by HIV or after an organ transplant. HIV is a virus that weakens the immune system.

If you've been diagnosed with HIV and have possible symptoms of Kaposi's sarcoma, your HIV specialist will usually arrange tests for you. Or you may go to your doctor with symptoms and be referred to a specialist for tests.

A specialist may suspect Kaposi's sarcoma just by looking at the skin lesions. But you'll need a biopsy to be sure.

If you're diagnosed with Kaposi's sarcoma and haven't been tested for HIV, your doctor and nurse will talk to you about having a test. Knowing whether you have HIV helps doctors decide how to treat the Kaposi's.

You may also have further tests to check whether the cancer has spread to other areas of the body. Your doctor will explain any tests you need.

In a skin biopsy, the doctor uses a small round cutting instrument to remove a small sample of skin. The sample is then looked at under a microscope.

Before the biopsy, the doctor will give you an injection of local anaesthetic to numb the area.

You can have the biopsy done in the outpatient department. The area may be sore for a few days, so you might need to take some painkillers.

The doctor may ask for a chest X-ray if they want to check whether Kaposi's sarcoma has spread to your lungs.

An endoscope is a thin, flexible tube with a camera at the end. Your doctor or specialist nurse may use it to look for Kaposi's sarcoma inside the body. During the procedure they can take biopsies of any abnormal areas.

An endoscopy can be used to look inside the lungs, and this is called a bronchoscopy. Or you might have an endoscope to look inside the gullet and stomach.

A CT scan takes a series of X-rays and puts them together to build a 3D picture of the inside of your body.

A team of specialists will meet to talk about the best treatment plan for you. This is called a multidisciplinary team (MDT).

Treatment of Kaposi's sarcoma depends on a few things, including:

• the type of Kaposi's you have
• the number and size of your lesions
• where the lesions are
• your general health

Treatment is usually given by doctors and nurses who specialise in the type of Kaposi's you have. Or they may be experts in the part of the body where the Kaposi's sarcoma is. This may include a:

• dermatologist, a specialist who treats skin conditions
• specialist in treating infectious diseases, like HIV
• oncologist, a cancer doctor

Your doctor and nurse will talk to you about the best treatment for you. They can also go through the things to think about when you're making treatment decisions.

Most people with Kaposi's sarcoma skin lesions start with treatment given directly to the lesions. This is called local treatment.

For early skin lesions, the most common treatment is a cream put straight onto the skin. It could be one of these:

• an immunotherapy cream called imiquimod (Aldara®), which is also used to treat some skin cancers and is the most common choice
• a cream called alitretinoin gel 0.1%
• a type of retinoid cream (like vitamin A), which is sometimes used

Local treatments help improve the lesions you already have. But they don't stop new lesions from developing.

Other treatments are sometimes used for skin lesions, but this isn't common.

For Kaposi's sarcoma, doctors sometimes use surgery to remove small skin lesions.

Cryotherapy treats Kaposi's sarcoma by freezing the cancer cells with liquid nitrogen, which destroys them.

In some cases, chemotherapy drugs are injected straight into the skin lesions of Kaposi's sarcoma. Doctors call this intralesional chemotherapy.

It can shrink the lesions and make them lighter in colour. Sometimes they disappear completely.

The side effects are swelling, pain and blistering around the area where the injection is given. This type of chemotherapy doesn't cause side effects in the rest of the body.

Radiotherapy may be used to treat the skin lesions of Kaposi's sarcoma. The dose is usually low, so it doesn't cause many side effects.

You may notice some skin changes in the treated area, and you may feel tired. Once the radiotherapy finishes, these side effects gradually improve.

Chemotherapy can treat Kaposi's sarcoma that is inside the body. It's given into a vein (intravenous) or as capsules. Doctors might also use it if you have lots of skin lesions over large areas of skin, or if the lesions break or bleed (become ulcerated).

You may have 1 of the following drugs:

• vinorelbine, which you'll take as capsules
• pegylated liposomal doxorubicin, usually every 3 weeks
• paclitaxel, every 2 to 3 weeks

Liposomal drugs have fewer side effects than standard chemotherapy. This means treatment can be given for longer.

Radiotherapy uses high-energy X-rays to destroy cancer cells, while doing as little harm as possible to normal cells. It isn't commonly used to treat Kaposi's sarcoma.

If Kaposi's sarcoma is affecting organs inside the body, radiotherapy might be used to improve symptoms like swelling, pain and bleeding. This is called palliative radiotherapy.

Your treatment will also depend on the type of Kaposi's sarcoma you have.

Before you decide on treatment for HIV-related Kaposi's sarcoma, you and your specialist doctor will look at a few things together:

• how well you are
• how well your immune system is working
• whether you have symptoms

If you have early-stage Kaposi's sarcoma and haven't had treatment for HIV, you'll usually start combination anti-retroviral therapy (CART).

This brings down the level of HIV in your body and improves your immunity. Once treatment starts, the Kaposi's sarcoma often gets better or goes away altogether. But this can take several months to happen.

If you have only a few skin lesions in one small area of your body, you can have the treatment applied straight to the lesions. This is called local treatment, and it uses creams.

Your doctor may offer you chemotherapy that treats the whole body if:

• Kaposi's sarcoma is more advanced, or
• you're already having HIV treatment when it's diagnosed

Chemotherapy is also used if the skin lesions are broken or bleeding (ulcerated). It's used too if your limbs are swollen, or if Kaposi's sarcoma is affecting the:

• lungs
• stomach
• bowel
• mouth

Classic or endemic Kaposi's sarcoma may not need treatment straight away. It grows slowly, and it may not cause any problems.

Chemotherapy or local treatments may be used if classic Kaposi's sarcoma:

• starts to grow or spread more quickly
• affects a number of areas

Kaposi's sarcoma that develops after a transplant can sometimes be brought under control by stopping the immunosuppressant drugs you're taking, or by changing them. If that doesn't work, you may need chemotherapy or radiotherapy.

You'll have regular check-up appointments at the hospital. Your doctor or specialist nurse will examine you and ask about any side effects or symptoms.

Always tell them about any new symptoms or side effects that are not getting better.

Coping with skin lesions can be difficult, especially if they're very visible. With Kaposi's sarcoma, you may be able to use skin camouflage to cover small, flat lesions and make them less noticeable.

You can learn how to apply camouflage make-up from Changing Faces. Some clinical nurse specialists can also give you advice about using it.

Kaposi's sarcoma can damage the lymph vessels, and when that happens, fluid can build up in the arms or legs. This is called lymphoedema.

Lymphoedema is a chronic swelling. That means it never goes away, because the cause can't be reversed. Some treatments can reduce the swelling, though.

Your doctor can refer you to specialist lymphoedema clinics for treatment.

Kaposi's sarcoma can change how your body looks, through skin lesions or lymphoedema, and that can affect how you think and feel about your body. If this is worrying you, talk to your nurse about it.

Even if you already live a healthy life, you may choose to make some positive changes after your cancer treatment. Small changes, like eating well and keeping active, can improve your health and wellbeing and support your body as it recovers.