Cancer of the vulva
Introduction
Cancer of the vulva is a rare type of cancer. There are about 1,000 diagnosed cases in the UK each year.
The symptoms of vulval cancer include pain, itching and bleeding of the vulva (see symptoms).
The vulva
The vulva is made up of the:
- outer part of the vagina,
- clitoris, and
- two pairs of lips surrounding the entrance to the vagina, called the labia majora (the outer pair) and the labia minora (the inner pair).
Types of vulval cancer
There are two main types of vulval cancer:
- primary vulval cancer, where the cancer begins inside the vulva, and
- secondary vulval cancer, where the cancer begins in another part of the body (usually the reproductive system), such as the cervix or ovaries, and then spreads to the vulva.
The rest of these pages will focus on primary vulval cancer. See selected links for more information about other cancers of the reproductive system.
Types of primary vulval cancer
There are five main types of vulval cancer. They are classified according to the type of cells that the cancer begins in:
- Squamous cell carcinoma is the most common type of vulval cancer, accounting for nine out of 10 cases. It develops from the squamous cells, the cells in the outer layer of skin. Squamous cell carcinoma usually grows slowly, over a few years.
- Vulval melanoma is the second most common type, but is still rare. It develops from the pigment-producing cells of the skin.
- Adenocarcinoma is very rare, developing from the cells that line the glands in the vulva.
- Verrucous carcinoma is a very rare and slow-growing type of vulval cancer that looks like a wart.
- Sarcoma is extremely rare, developing in tissue such as muscle or fat under the skin.
Cancer may occur in any part of the vulva. The most common sites are the edges of the inner and outer lips.
Less often, vulval cancer may affect the clitoris or the two small glands either side of the vagina called the Bartholin's glands. Occasionally, it is diagnosed in the skin that divides the vulva from the anus (the perineum).
Who is affected?
Most cases of vulval cancer are in women aged over 60. Early-stage vulval cancer (which is curable) can ocassionally occur in young women in their 20s, but it is extremely rare for these women to have later-stage vulval cancer.
Outlook
The outlook for vulval cancer is generally good if the cancer has not spread beyond the vulva.
However, the outlook for the melanoma type of vulval cancer is poor because it is very aggressive and spreads quickly.
The main treatment for vulval cancer is surgery to remove the cancerous tissue. Radiotherapy and chemotherapy may also be given (see treatment).
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Symptoms
Vulval cancer does not develop quickly. First, there is a change in cells, as normal cells become abnormal. This is known as the pre-cancerous stage, and does not mean that you have cancer or that cancer will necessarily develop. If abnormal cells are found early on, pre-cancerous changes can be treated before they become invasive cancer of the vulva.
The symptoms of pre-cancerous cell change and vulval cancer are similar and may include the following:
- itching, pain or soreness of the vulva
- thickened, raised, red, white or dark patches on the skin of the vulva,
- an open sore or growth (which may bleed) on the skin of the vulva
- burning pain when you pass urine,
- a mole on the vulva that changes shape or colour, and
- general pain of the vulva.
Some of the above can also be symptoms of less serious conditions such as thrush. However if you have any of these symptoms, you should see your doctor.
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Causes
How does cancer begin?
Cancer begins with a change in the structure of DNA, which is found in all human cells. DNA provides cells with a basic set of instructions, such as when to grow and when to reproduce.
A change in the DNA's structure (genetic mutation) changes these instructions so that the cells carry on growing and reproducing uncontrollably. This produces a lump of tissue known as a tumour.
How does cancer spread?
Left untreated, cancer can quickly grow and spread to other parts of the body, usually through the lymphatic system.
The lymphatic system is a series of glands (or nodes) located throughout your body. It is similar to the blood circulatory system. Lymph glands produce many specialised cells that are needed by your immune system to fight infection.
Once the cancer reaches the lymphatic system, it can spread to any other part of the body, including your bones, blood and organs.
Risk factors
The exact cause of vulval cancer is unknown. However, evidence suggests that a number of risk factors can increase your likelihood of developing vulval cancer:
- human papilloma virus (HPV),
- vulval intraepithelial neoplasia (VIN),
- vulval skin conditions, and
- smoking.
Human papilloma virus (HPV)
Human papilloma virus (HPV) is the name given to a family of viruses that affect the skin and the moist membranes that line the body, such as those in the cervix, anus, mouth and throat.
There are many different types of HPV, some of which are more high risk than others.
HPV DNA has been found in up to 80% of samples of cancerous tissue, which suggests that HPV may increase your risk of developing vulval cancer.
HPV is known to cause changes in the cells of the cervix, which can lead to cervical cancer. It is thought that the virus could have a similar effect on the cells of the vulva.
HPV is spread during sexual intercourse, including anal and oral sex.
Vulval intraepithelial neoplasia (VIN)
A condition called VIN (vulval intraepithelial neoplasia) can occur in the skin of the vulva, and is linked to infection by HPV.
VIN is a form of pre-cancerous changes in the skin of the vulva. Occasionally, the cells change from normal to abnormal, with the potential to become cancerous.
The most common symptom of VIN is a persistent itch, along with thickened, swollen or discoloured patches of affected skin. Women with the condition should be treated or closely monitored by a doctor.
Vulval skin conditions
Women who have certain long-term, non-cancerous skin conditions, such as vulval lichen sclerosus and vulval lichen planus, have an increased risk of developing cancer of the vulva. However, this risk is still small: only a small percentage (1-2%) of women who have lichen sclerosus will go on to develop vulval cancer.
The most common symptoms of vulval lichen sclerosis and lichen planus are itchy and sore, discoloured skin around the area, which may split, causing stinging and pain. Specially prescribed ointment can control symptoms, and you will need to see your doctor regularly.
Smoking
Cigarette smoking increases your risk of developing VIN and vulval cancer, possibly because smoking damages your immune system.
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Diagnosis
Screening
Because vulval cancer is so rare, there is currently no programme in place to screen for the cancer. However, an examination of the vulva is carried out during routine cervical smear tests. Pre-cancerous conditions can be identified during this examination, and treated early on to prevent vulval cancer developing.
Self-examining
It is a good idea to check your own vulva regularly to detect any changes to the skin. Using a mirror, you can look for areas that are red, irritated, white or darkly coloured. You will also be able to see any growths, nodules or sores. If you do notice any changes like this, see your doctor.
Before diagnosing vulval cancer, your GP will ask you about your symptoms and look at your medical history to see what illnesses you have had in the past.
Your vulva will be examined to see if there are any lumps or unusual areas of skin. In nine out of every 10 women diagnosed with vulval cancer, their doctor will be able to see a lump or sore area.
Referral to a gynaecologist
If no obvious cause of your symptoms can be found, your GP will probably refer you to a gynaecologist for further testing. A gynaecologist is a specialist in treating conditions of the female reproductive system.
Colposcopy and biopsy
Gynaecologists often use an instrument called a colposcope to look for any abnormalities in the vulva, cervix and vagina.
A colposcope is a small microscope with a light on the end of it. During the examination (colposcopy), a tissue sample will also be taken (biopsy) from the place where the cancer might be, using a special instrument.
Anesthetic cream is applied to your vulva and an injection of local anaesthetic is given, so the biopsy should not hurt. You may have slight bleeding and soreness afterwards.
The tissue sample can then be checked in a laboratory to find out whether you have cancer, and what type of cancer it is. Your doctor will usually see you about seven to 10 days later, when the results come through.
Further testing
If the results of the biopsy suggest that you have cancer, and there is a risk that the cancer may have spread, you will probably require further testing to assess how widespread the cancer is.
These tests may include:
- Cystoscopy: This is an examination of the inside of the bladder. It is done to see if the cancer has spread to the bladder. A thin, hollow viewing tube is inserted into the bladder to look for tumours and take a tissue sample.
- Proctoscopy: This is an examination of the inside of the rectum. The doctor either uses a gloved finger on an instrument called a proctoscope to check for abnormalities such as growths on the rectal wall.
- CT scan: This is a computerised X-ray that can take detailed pictures of the inside of the body. It is useful for showing up cancer tumours, and checking whether cancer cells have spread.
- MRI scan: This is a scan that uses radio waves and magnets to produce detailed pictures of the inside of the body. It is used to check whether cancer has spread.
- X-ray: You may have a chest X-ray to check that cancer has not spread to your lungs.
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Treatment
Cancer treatment team
Many NHS Trusts have multi-disciplinary teams that treat vulval cancer. See below.
If you have vulval cancer, you may see several or all of these professionals as part of your treatment.
Deciding what treatment is best for you can be difficult. Your cancer team will make recommendations, but the final decision will be yours.
Before going to hospital to discuss your treatment options, you may find it useful to write a list of questions to ask the specialist. For example, you may want to find out what the advantages and disadvantages of particular treatments are.
Staging
Health professionals use a staging system to describe how far vulval cancer has spread.
- Stage 0. This is very early cancer found only in the surface of the skin of the vulva. It is sometimes called carcinoma in situ, or Bowen's disease.
- Stage 1. The cancer is only in the vulva and/or the perineum (skin between the rectum and vagina). The cancer is no more than 2cm wide.
- Stage 2. The cancer is only in the vulva and/or perineum and is larger than 2cm.
- Stage 3. The cancer has spread into nearby tissues such as the anus or vagina. It may have spread to nearby lymph nodes.
- Stage 4. The cancer has spread to the lymph nodes on both sides of the groin or to the urethra (the tube through which you urinate), bladder or bowel.
Treatment of vulval cancer depends on the size of the cancer, whether it has spread to other parts of the body, and your general health.
Early-stage vulval cancer can usually be cured with surgery (see below). But if vulval cancer is at an advanced stage and has spread to other organs, a cure may not be possible. In this situation, radiotherapy and chemotherapy can relieve the symptoms and slow down the spread of cancer.
Your doctor should tell you about the risks, benefits and side effects of the treatments being offered, and how they may affect your life afterwards. The treatments you may be offered are described below.
Surgery
Surgery is the main treatment for cancer of the vulva. If the cancer is quite small, your doctor is likely to recommend an operation called wide local excision. The aim is to remove all of the cancer and for extra safety, some of the healthy tissue around it.
If the cancer is bigger, it is more likely to have spread to the lymph nodes. As well as removing the cancer from the vulva, your doctor may want to take out the lymph nodes in the groin, to try to stop the cancer spreading around your body. Rarely, the lymph nodes in the pelvis may be taken out too.
Your surgeon will not remove more tissue than is necessary, but if the cancer is quite large, you may need to have quite a lot of tissue removed. If your labia has to be removed (the folds of skin around your vagina) the operation is called a radical vulvectomy.
If only a small amount of tissue has been removed, the skin of the vulva will be neatly stitched together. Otherwise, it may be necessary to have a skin graft, where a piece of skin is taken from your thigh or abdomen to cover the wound in your vulva.
Radiotherapy
If your lymph nodes show signs of cancer, you are likely to have radiotherapy after your operation. The aim is to destroy any cancer cells left behind after surgery.
Radiotherapy uses radiation to kill cancer cells. It is usually given from outside the body using a machine that directs high energy X-rays at the cancer. This is called external beam radiotherapy.
Rarely, if a patient is not well enough for an operation, radiotherapy may be offered instead of surgery.
Radiotherapy can also be used to help patients whose cancer cannot be cured. It can help to relieve symptoms and prolong a good quality of life.
While it kills cancerous cells, radiotherapy can also affect healthy tissue and has a number of side effects, including:
- sore, red skin (like sunburn),
- pain while passing urine,
- loss of taste,
- loss of appetite,
- tiredness, and
- nausea.
If you have external radiotherapy to the pelvis, you may experience an early menopause (if you have not had the menopause already). This means you will no longer be able to have children.
Chemotherapy
Chemotherapy is usually used in combination with radiotherapy or to control symptoms when a cure is not possible (called palliative chemotherapy).
Chemotherapy is the use of anti-cancer drugs to destroy cancer cells. It is usually given by injection (called intravenous chemotherapy).
Like radiotherapy, the powerful medicines used in chemotherapy can also damage healthy tissue and cause a range of side effects. Side effects of chemotherapy include:
- nausea,
- vomiting,
- hair loss, and
- fatigue.
Chemotherapy can also weaken your immune system, making you more vulnerable to infection. However, the side effects should stop once treatment has finished.
Clinical trials
As vulval cancer is rare, you may be asked to take part in a clinical trial. Clinical trials are an important way for health professionals to learn more about the best way to treat specific conditions.
Most clinical trials involve comparing a new treatment with an existing treatment to determine whether the new treatment is more or less effective.
If you do receive a new treatment, there is no guarantee that it will be more effective than an existing one.
Your care team can tell you if there are clinical trials in your area, and explain the advantages and disadvantages of taking part.
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Prevention
Practising safe sex
There is a strong link between certain types of human papilloma virus (HPV) and the development of abnormalities that may turn into vulval cancer.
As HPV is spread through unprotected sex, using a condom is the best way to avoid it.
Before beginning a sexual relationship with a new partner, it is a good idea for you both to be tested for sexually transmitted infections at a sexual health (GUM) clinic. All tests are free and confidential.
Cervical smear tests
Regular cervical smear tests are also important in detecting sexually transmitted infections and pre-cancerous cell changes early on.
HPV vaccination
There is now a vaccine that provides protection against the strains of HPV that are thought to be responsible for most cases of vulval cancer. HPV vaccination also protects against cervical cancer, which is far more common than vulval cancer.
Girls should be offered the HPV vaccine as part of their routine childhood immunisation programme. The vaccine should be given to girls who are 12 to 13 years old, with three doses given over six months.
Quit smoking
You can decrease your chance of getting vulval cancer by not smoking. Smokers are less able to get rid of the HPV infection from the body, which can develop into cancer.
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