CANCER OF VULVA
( By JASCAP )

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Causes & Diagnosis

Causes of vulval cancer

Cancer of the vulva is rare: each year just over 1000 women in the UK are diagnosed with it. Research is going on all the time into the cause of vulval cancer. The following have been identified as things that might increase the risk that people will develop cancer of the vulva:

Human papilloma virus (HPV) This is an infection that can be associated with vulval cancer. HPV is passed between people during sex and there are many different types. Some types, such as types 6 and 11 can cause genital warts, but are not usually associated with the development of cancer. Others are considered more likely to cause cancer and these include HPV types 16, 18 and 31. These types can lead to pre-cancerous changes called VIN.

Vulval intraepithelial neoplasia (VIN) is a pre-cancerous condition that can occur in the skin of the vulva, and is linked to HPV infection. There are three levels of abnormality − VIN1, VIN2 and VIN3. VIN3 is the most abnormal, and in some women can develop into cancer of the vulva if it is not treated. Almost one-third of vulval cancers develop in women who have VIN.
Vulval skin conditions Women who have certain non-cancerous skin conditions of the vulva over a long period of time, have a higher risk of developing cancer of the vulva. The conditions are called vulval lichen sclerosus and vulval lichen planus. They can cause long-term inflammation of the skin in the vulval area. Almost two-thirds of vulval cancers occur in women who have lichen sclerosus. But it is important to remember that only 1−2% (1Ė2 in 100) of women who have lichen sclerosus will go on to develop vulval cancer. We can send you information about lichen sclerosus and lichen planus.

Paget's disease This is a rare type of cancer of the vulva which can sometimes be linked with a cancer elsewhere in the pelvic area or the digestive system.

Smoking Cigarette smoking may increase the risk of developing both VIN and vulval cancer. This may be because it can make the immune system work less effectively.

Like other cancers, cancer of the vulva is not infectious and canít be passed on to other people. It is not caused by an inherited faulty gene and so other members of your family are not likely to be at risk of developing it.

Symptoms of vulval cancer

The most common symptoms of cancer of the vulva are:

itching, burning and soreness of the vulva
a lump, swelling or wart-like growth
thickened, raised, red, white or dark patches on the skin of the vulva
bleeding or a blood-stained vaginal discharge
burning pain when passing urine
pain in the area of the vulva
a sore or ulcerated area on the vulva
a mole on the vulva that changes shape or colour.

Any of these symptoms can be due to conditions other than cancer, but always get your doctor to check them.

Cancer of the vulva can take many years to develop as it usually grows slowly. As with other cancers, it is easier to treat and cure if it is diagnosed at an early stage.

Types of vulval cancer

Squamous cell carcinoma

Nine out of ten (90%) of cancers of the vulva develop from squamous cells in the outer layer of the vulva. Squamous cell cancers usually grow very slowly over a few years.

Vulval melanoma

Melanomas develop from the pigment-producing cells that give the skin its colour. Vulval melanoma is the second most common type of vulval cancer, but is much less common than the squamous cell type. Only about 4 out of every 100 (4%) vulval cancers are melanoma.

Adenocarcinoma

These are very rare. Adenocarcinoma of the vulva develops from cells that line glands in the vulval skin. Pagetís disease of the vulva is a condition where adenocarcinoma cells spread out from these glands and across the skin of the vulva.

Verrucous carcinoma

Verrucous carcinoma is a rare, very slow-growing type which looks like a large wart.

Sarcomas

Sarcomas of the vulva are extremely rare. Sarcomas develop from cells in tissue such as muscle or fat under the skin. They tend to grow more quickly than other types of cancer. Several different types of sarcoma can affect the vulva, including:

leiomyosarcomas and rhabdomyosarcomas (which develop from muscle cells)
angiosarcomas (from blood vessels)
neurofibrosarcomas (from nerve cells)
epithelioid sarcomas (from cells in the skin).

Diagnosis of vulval cancer

Usually you begin by seeing your GP, who will examine you. If there is any possibility of you having vulval cancer you should be referred to a gynaecological cancer specialist (specialist in womenís cancers). Your doctor may also arrange for you to have a blood test and chest x-ray to check your general health.

At the hospital the gynaecologist will take your medical history and will examine your vulval area. They may also carry out some tests. The nurse will help you to position yourself on a couch that has special leg supports. Understandably, some women find this kind of examination embarrassing or upsetting. If you feel like this let your doctor or nurse know so that they can support you emotionally.

To examine your vulva the doctor may use a colposcope. This helps to identify any abnormal areas. The colposcope is like a small microscope with a bright light that can magnify areas so that the cells can be seen more clearly. The colposcope stays outside the body. A biopsy will be taken.

You will also have an internal examination to check your vagina and cervix for any abnormality. The doctor will use a speculum (a plastic or metal instrument) to hold the vaginal walls open. A liquid is then dabbed on to the cervix to make any abnormal areas show up more clearly. A colposcope is used to examine the cervix. Sometimes a small sample of cells from the cervix will be taken. Some women with lichen planus or lichen sclerosus can have narrowing of the vagina. In this situation it may be necessary to have the sample of cells taken under a general anaesthetic.
The doctor may also examine your back passage (anus) to check for any abnormal lumps. The skin around your back passage may be examined.

Biopsy

This procedure can usually be done in the outpatients clinic. Anaesthetic cream is applied to the vulva to numb the area, and then an injection of local anaesthetic is given. The doctor takes the biopsy (a small amount of cells) from the abnormal area using a biopsy instrument. The biopsy is examined under a microscope by a pathologist. This is the best way of diagnosing cancer of the vulva. Because the local anaesthetic numbs the area there should be no pain, but you may feel a little discomfort during the biopsy. Afterwards, you may have slight bleeding (let your doctor know if it is more than this), and slight soreness. The soreness can be relieved with mild painkillers and a warm bath. The doctor will usually see you about 7Ė10 days after the biopsy to give you the results. Waiting for the results can be a stressful time and support organisations can help you to cope.

Further tests for vulval cancer

After a diagnosis is made, further tests are often needed to find out about the size and position of the cancer, and whether it has spread. This process is called staging, and may take some time. The results will help you and your doctor to decide on the best treatment for you. These tests may be done again (during treatment, or when treatment ends) to check the effectiveness of the treatment. Although tests are useful, no one test can diagnose a vulval cancer. Occasionally other medical conditions can give similar results, making it difficult to decide what is and is not cancer. Doctors piece together information from different tests and examinations, along with your symptoms and medical history. They then put all this information in context.

The following tests are most often used with cancer of the vulva.

Blood test
Chest x-ray
CT (CAT) scan
Magnetic resonance imaging (MRI scan)
Examination under anaesthetic (EUA)

Blood test

A sample of your blood is taken to check your general health, the number of cells in your blood (blood count) and to see how well your kidneys and liver are working.

Chest x-ray

This is taken to check that your lungs and heart are healthy.

CT (CAT) 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 to 30 minutes. 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.

Magnetic resonance imaging (MRI scan)

This test is similar to a CT scan but uses magnetism instead of x-rays to build up a detailed picture of areas of your body.

During the test you will be asked to lie very still on a couch inside a long tube for about 30 minutes. It is painless but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It is also noisy, but you will be given earplugs or headphones. Some people are given an injection of dye into a vein in the arm, but this usually does not cause any discomfort. It will probably take several days to get the results of your tests, and this waiting period is likely to be a worrying time for you.

Examination under anaesthetic (EUA)

This is an examination of the vulva carried out under a general anaesthetic. It allows the doctor to examine you thoroughly without causing any discomfort and to check the extent of the cancer.

Staging and grading of vulval cancer

The stage of a cancer is a term used to describe its size and whether it has spread beyond its original area of the body. Grading refers to the appearance of cancer cells when they are looked at under the microscope. The grade gives an idea of how quickly the cancer may develop. Knowing the extent of the cancer and the grade helps the doctors to decide on the most appropriate treatment.

Staging
Grading

Staging

A commonly used staging system is described below:

Stage 0 or carcinoma in situ This is very early cancer (some doctors describe it as pre-cancer). The cancer is found in the vulva only and is only in the surface of the skin.

Stage 1 Cancer is found only in the vulva and/or the space between the opening of the rectum and the vagina (perineum).The affected area is 2 cm (about 1 inch), or less, wide.

Stage 2 Cancer is found in the vulva and/or the perineum. The affected area is larger than 2 cm (1 inch).

Stage 3 Cancer is found in the vulva and/or perineum and has spread to nearby tissues such as the lower part of the urethra (the tube through which urine passes), the vagina, the anus (the opening of the rectum) and/or nearby lymph nodes.

Stage 4 The cancer has spread beyond the urethra, vagina and anus into the lining of the bladder or the bowel; or, it may have spread to the lymph nodes in the pelvis or to other parts of the body.

Grading

There are three grades; grade 1 (low-grade), grade 2 (moderate-grade) and grade 3 (high-grade).

Low-grade means that the cancer cells look very much like the normal cells of the vulva. They usually grow slowly and are less likely to spread.
Moderate-grade means the cells look more abnormal than low-grade cells but not as abnormal as high-grade cells.
High-grade means the cells look very abnormal. They are likely to grow more quickly and are more likely to spread.

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