Exactly what causes the cells of the vulva to become cancerous is unclear although there are a number of things known to increase the risk of developing vulval cancer. 

These are:

• age – around three out of four cases of vulval cancer develop in women aged 65 or over
• human papilloma virus (HPV)
• vulval intraepithelial neoplasia (VIN)
• skin conditions that can affect the vulva, such as lichen sclerosus (see below)
• 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.

HPV is known to cause changes in the cells of the cervix, which can lead to cervical cancer. It’s also thought that the virus could have a similar effect on the cells of the vulva. One expert has estimated that around 30% of vulval cancer cases are associated with an HPV infection.

The HPV virus is spread during sexual intercourse, including anal and oral sex and is thought to be one of the leading causes of vulval cancer in younger women.

Vulval intra-epithelial neoplasia (VIN)

Vulval intra-epithelial neoplasia is what’s known as a pre-cancerous condition.

This means there are changes to the biological make-up of certain cells which are not the same as new cancers but could potentially lead to the formation of a new cancer at a later date.

Symptoms of vulval intra-epithelial neoplasia are similar to that of vulval cancer and include:

• persistent itchiness in the vulva
• a burning sensation when passing urine
• raised patches of skin on in the vulva that can be red or white

There are two types of vulval intra-epithelial neoplasia (VIN):

• usual VIN or undifferentiated VIN – this usually affects women aged 35 to 55 and is thought to be caused by an HPV infection
• differentiated VIN or dVIN – this is a rarer type, usually affecting older women aged 55 to 85 and is associated with skin conditions that affect the vulva (see below)

VIN can be treated by removing affected areas of skin or, in some cases, using a cream to help kill abnormal cells.

For more information, see:

• Cancer Research UK – current state of research into vulval cancer
• the British Society for the Study of Vulval Disease (BBSVD) – Vulval intra-epithelial neoplasia (VIN)

Skin conditions

Several skin conditions can affect the vulva, two of them being lichen sclerosus and lichen planus .

Both conditions cause similar symptoms – they cause affected areas of skin to become very itchy and sore and lead to the development of a red raised rash.

The causes of both conditions are unknown.

It is estimated that around 3-5% women who develop one of these conditions will go on to develop vulval cancer.

Smoking

Smoking increases your risk of developing vulval cancer.

One study found that women who smoked were twice as likely to develop vulval cancer than non-smokers.

The chemicals in tobacco smoke are known to cause damage to the cells of the body. Smoking can also weaken your immunity (defence against infection), making you more vulnerable to the harmful effects of a HPV infection.

How cancer develops

Cancer begins with a change in the structure of DNA. DNA provides our cells with a basic set of instructions, such as when to grow and reproduce.

A change in DNA structure is known as a mutation, and it can alter the instructions that control cell growth.

This means that the cells continue to grow instead of stopping when they should, producing a lump of tissue called a tumour.

How vulval cancer spreads

There are three ways that vulval cancer can spread:

• directly – spreading out of the tissue of the vulva and into surrounding parts of the body such as into the inside of the vagina or urethra
• via the lymphatic system –  the lymphatic system is a series of nodes (or glands) and channels that are spread throughout your body, much like your blood circulation system (see below)
• via the bloodstream into distant organs such as the lungs, brain, liver and bones – this generally only happens in advanced vulval cancer after the cancer cells have spread to multiple lymph nodes

Cancer that has spread to other parts of the body is known as metastatic cancer.

The emotional impact of living with vulval cancer can be significant. Many people report experiencing a kind of roller-coaster effect.

You may feel down at receiving a diagnosis, feel up when the cancer has been removed from your body and then feel down again as you try to come to terms with the after-effects of surgery.

This can sometimes trigger feelings of depression. Signs that you may be depressed include:

• feeling down or hopeless
• no longer take pleasure in things you enjoy

If you think you may be depressed, contact your GP for advice. There are a range of successful treatments for depression such as antidepressant medication and talking therapies such as cognitive behavioural therapy.

Read more about depression and coping with cancer.

Due to its rarity there are no dedicated charities or support groups for women with vulval cancer but you may find it useful to contact one of the main cancer charities such as:

• Macmillan Cancer Support – who have a helpline on 0808 808 00 00 (Monday to Friday, 9am to 8pm)
• Cancer Research UK – who have a cancer nurse helpline on 0808 800 4040 (Monday to Friday, 9am to 5pm)

Complications of vulval cancer surgery

Having the lymph nodes in your groin removed can sometimes disrupt your lymphatic system.

One of the functions of the lymphatic system is to drain away excess fluid from your body, so removing them can lead to a build-up of fluid in the tissue. 

This in turn can cause certain body parts to swell; in this case the arms and legs. This condition is known as lymphoedema.

There are exercises and massage techniques you can be taught to help reduce the swelling. Wearing specially designed bandages and compression garments can also help.

Read more about the treating lymphoedema.

Before examining your vulva, your GP will ask you about your symptoms and look at your medical history.

Your vulva will be examined for any lumps or unusual areas of skin. If you would prefer to be examined by a female doctor or you would like a nurse present during the examination, let your GP’s surgery know in advance of your appointment.

Referral to a gynaecologist

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 a small microscope with a light on the end of it (a colposcope) to look for any abnormalities in the vulva, cervix and vagina. This is known as a colposcopy.

During the colposcopy, a tissue sample will also be taken if cancer is suspected. This is called a biopsy.

A local anaesthetic is given, so the biopsy should not hurt, although you may have slight bleeding and soreness afterwards.

The tissue sample is checked in a laboratory for cancer. Your doctor will usually see you up to ten days later, when the results come through.

You may have additional biopsies to remove tissue from the lymph nodes near your vulva. This is to check whether cancer has spread through your lymphatic system.

Further testing

If the results of the biopsy show cancer and there is evidence it may have spread, you will probably need further tests to assess how widespread it is.

These may include the following tests, described below.

• Cystoscopy – an examination of the inside of the bladder to see if the cancer has spread to the bladder. A thin, hollow tube is inserted into the bladder to look for tumours and take a tissue sample.
• Proctoscopy – 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 wall of the anus.
• CT scan – a computerised X-ray that takes 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 – 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.

Staging

Staging in vulval cancer is based on the findings from surgery and is an assessment of whether the cancer has spread. The lower the stage, the greater the chance of a complete cure.

The staging system for vulval cancer is:

Stage 1 – the cancer is confined to the vulva. Stage 1 is further divided into two sub-types:

• Stage 1A – the cancer is 2cm or less in size
• Stage 1B – the cancer is larger than 2cm in size

Stage 2 – the cancer has spread beyond the vulva to other nearby parts of the body such as the lower vagina, anus or lower urethra (the tube you urinate out of), but the lymph nodes are unaffected.

Stage 3 – the cancer has spread into nearby lymph nodes. Stage 3 is divided into three sub types:

• Stage 3A – the cancer has spread into one lymph node and the metastasis is 5mm or more in size OR the cancer has spread into one or two lymph nodes but each metastasis is less than 5mm
• Stage 3B – the cancer has spread into two or more lymph nodes and the metastases are each 5mm or more in size OR the cancer has spread into three or more lymph nodes but each metastatis is less than 5mm
• Stage 3C – the cancer has spread through the outer covering of at least one of the lymph nodes (extracapsular spread)

Stage 4 – the cancer has spread away from the vulva and perineum (the area of skin and tissue between the anus and the genitals) to other parts of the body. Stage 4 is divided into two sub types:

• Stage 4A – the cancer has spread into the upper urethra or lining of the vagina, or into the bladder, rectum or the bones of the pelvis OR the cancer has caused lymph nodes to become stuck to underlying tissue or caused open sores (ulcers) to develop in the groin
• Stage 4B – the cancer has spread to more distant parts of the body such as the lungs, liver or the lymph nodes that are contained in the pelvis

Stage 1 and 2 vulval cancers are regarded by most experts as early stage cancers with a relatively good chance of a complete cure.

Stage 3 and 4 vulval cancers are usually regarded as advanced-stage cancers and a cure for these types of cancers may not always be possible.

Cancer of the vulva is a rare type of cancer with less than 1,200 new cases being diagnosed each year in the UK.

Symptoms of vulval cancer include:

• the development of a noticeable lump, mass or wart-like growth on a section of the vulva
• persistent itchiness in the vulva
• pain when passing urine
• bleeding from the vulva or passing a blood-stained discharge

Read more about the symptoms of vulval cancer.

Contact your GP if you experience any of these symptoms. They’re unlikely to be the result of vulval cancer, but they will require further investigation.

Types of vulval cancer

The various types of vulval cancer are classified by the type of cells the cancer first develops in.

By far the most common is known as squamous cell carcinoma, which accounts for over 90% of all cases of vulval cancer. In this type the cancer develops in cells found on the outer layer of the body.

Vulval melanoma accounts for 4% of all cases. In this type the cancer develops in the cells that give skin its colour.

Other very rare types of vulval cancer include:

• adenocarcinoma, which develops from the cells that line the glands in the vulva
• verrucous carcinoma, which is a slow-growing type of vulval cancer that looks like a wart
• sarcoma, which develops in tissue such as muscle or fat under the skin

Who is affected

There are two main groups of women affected by vulval cancer.

The largest group are older women over the age of 65 who often have a history of non-cancerous skin conditions affecting their vulva, particularly a condition called lichen sclerosus. This group account for up to four out of five cases.

The second, smaller group are younger women who have not yet gone through the menopause.

The exact cause of vulval cancer is still unclear but significant risk factors include:

• age
• smoking
• persistent infection with specific high-risk strains of the human papilloma virus (HPV) – particularly a strain known as HPV 16, which is a strain of HPV virus responsible for almost all cases of cervical cancer
• vulval intraepithelial neoplasia (VIN) – where the skin cells around the vulva experience pre-cancerous changes but “full blown” cancer has not yet developed; a minority of cases will go on to develop vulval cancer

HPV infection and VIN are responsible for the majority of cases in younger women.

Read more about the causes of vulval cancer and preventing vulval cancer.

Treatment

The main treatment for vulval cancer is surgery to remove the cancerous tissue from the vulva and to assess the lymph nodes in the groin to check if the cancer has spread.

A combination of radiotherapy and chemotherapy is used if it looks as though surgery would lead to a loss of bladder control (urinary incontinence) or bowel control (bowel incontinence). This combination is also used to slow the spread of advanced cancer if a cure is not possible, which is known as palliative care.

Read more about the treatment of vulval cancer.

Emotional impact

Any type of cancer is likely to have a considerable negative emotional impact. But developing a cancer in one of the most intimate parts of the body can be profoundly distressing for some woman.

It can also take several months to adjust to the effects of surgery – both physical and psychological.

There are a range of services that provide both practical and emotional support for people affected by vulval cancer. Read more in recovering from the effects of vulval surgery.

Outlook

The outlook for the squamous cell carcinoma type of vulval cancer is good and many women recover fully. Around 70% of women survive for at least five years after diagnosis, many will live much longer and most of them will be cured.

Squamous cell carcinomas diagnosed in the initial stages have a much higher five-year survival rate (90%) than carcinomas diagnosed in their most advanced stage (20%).

The outlook for the melanoma type of vulval cancer isn’t as good. But it is difficult to estimate reliable five-year survival rates as it is such a rare type of cancer.

There is a strong link between certain types of human papilloma virus (HPV) and the development of abnormalities that may turn into vulval cancer.

Practising safer sex

As HPV is spread through unprotected sex, using a condom is the best way to avoid it. However, a condom can only provide limited protection against HPV so it is still important to attend your cervical screening tests (see below) even if you do practise safer sex.

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 carried out in confidence.

Read more about safer sex and sexual health.

Cervical screening tests

Regular cervical screening tests are also important in detecting sexually transmitted infections and pre-cancerous conditions such as vulval intra-epithelial neoplasia.

Read more about on cervical screening tests.

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.

Read more about HPV vaccination.

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.

Read more about quitting smoking.

When recovering from surgery for vulval cancer, you may experience numbness and changes in sensation around your vulva. These feelings should pass within a few months.

Depending on the type of surgery you had, the appearance of your vulva may have changed quite a bit.

Some women are not particularly bothered by this. Other women can find these changes in appearance deeply upsetting.

If you are concerned about looking at your vulva for the first time you may want to do it in the company of a nurse who will be able to explain the changes.

The first few days

When you wake up after surgery it is likely that you will have a drip in your arm to provide you with fluids.

Usually a thin tube known as a catheter is inserted into your bladder in order to drain urine out of it. This can usually be removed after a few days.

You may also have a tube in your groin to drain away excess fluid as a result of your lymph nodes being removed. This can usually be taken out after a week.

The site of the surgery will usually be sealed with disposable stitches that should disappear once the wound heals.

You are likely to be in some pain for the first few days after the operation and need painkilling medication, which can be given as a injection, in tablet form or pumped directly into your spine. This is known as an epidural.

You will be encouraged to perform some gentle exercise as soon as possible after the surgery as this can help prevent problems such as blood clots in your legs (deep vein thrombosis).

A nurse or physiotherapist will show you what types of exercises are suitable for you.

Going home

Depending on the type of surgery you have it may take anywhere from a couple of days to a few weeks before you are well enough to leave hospital.

You may be told not to lift any heavy objects as this could damage your wound. You may also be told to avoid driving for four to six weeks and it may be a number of months before you are well enough to return to work. 

Sex

It is normally safe to have sex around six to eight weeks after surgery, but many women do not feel ready for sex even after this time.

Both the stress of living with vulva cancer and the impact of surgery can have a obvious impact on your feelings about sex that may take some time to comes to terms with.

You can read more information about sexuality and vulval cancer on the Macmillan Cancer Support website.

The most common initial symptoms of vulval cancer are a persistent itch in the vulva and a lump, swelling or mass in the vulva. In most cases this will be on or near one of your labia.

Other symptoms of vulval cancer include:

• raised and thickened patches of skin that can be red, white or black
• bleeding from your vulva or releasing a blood-stained discharge between periods
• a mole on the vulva that changes shape or colour
• pain and discomfort in your vulva
• a burning pain when you pass urine

When to seek medical advice

Contact your GP if you experience any of the symptoms listed above.

While it is highly unlikely to be the result of vulval cancer, these types of symptoms should be investigated further.

Treatment for vulval cancer usually involves a combination of surgery, radiotherapy and chemotherapy.

Your treatment plan

Deciding what treatment is best for you can often be confusing. Your cancer team will recommend what they think is the best treatment option, but the final decision will be yours.

Before visiting hospital to discuss your treatment options, you may find it useful to write a list of questions that you would like to ask the specialist. For example, you may want to find out the advantages and disadvantages of particular treatments.

Surgery to remove vulval cancer

In most cases your treatment plan will involve some form of surgery. The type of surgery will depend on the stage of the cancer.

There are three surgical options to treat vulval cancer:

• the cancerous tissue from your vulva is removed as a well as a margin of healthy tissue, usually around 1cm, as a precaution – radical wide local excision
• a larger section of your vulva is removed, such as one or both of the labia and the clitoris – radical partial vulvectomy
• this involves removing the whole vulva, including the inner and outer labia, and possibly the clitoris – radical vulvectomy

The time it will take you to recover from surgery will depend on the type of surgery and how extensive it was. For extensive operations such as a pelvic exenteration (see below) it may take up to eight weeks to recover.

Read more about recovering from the effects of surgery.

Assessing and removing groin lymph nodes

An additional surgical procedure is usually required to assess whether the cancerous cells have spread into one or more lymph nodes in your groin.

Sentinel node biopsy

In some circumstances this can be done during an operation to remove certain lymph nodes, known as sentinel nodes.

Sentinel nodes are identified by injecting a dye at the site of the tumour and studying its flow to locate the nodes closest to the tumour. These are then removed and checked for cancerous cells.

Groin lymphadenectomy

Depending on the results of the sentinel node biopsy, some or all of the nodes in your groin will need to be surgically removed. This type of operation is known as a lymphadenectomy.

With larger vulval tumours and in cases where the lymph nodes appear enlarged due to metastases, sentinel node biopsy is not suitable. Instead, the removal of all the lymph nodes in your groin is likely to be recommended. This is called a groin or inguino-femoral lymphadenectomy. 

If lymph nodes are affected by cancer, further treatment with radiotherapy is likely to be advised.

Performing a lymphadenectomy does reduce the risk of the cancer returning but it can make you more vulnerable to infection and cause swelling in your legs due to a build-up of lymphatic fluid. This type of swelling is known as lymphoedema. Read more about the complications of surgery for vulval cancer.

Pelvic exenteration

In cases of advanced vulval cancer or if the cancer returns after previous treatment, an operation called a pelvic exenteration may be recommended. This involves removing your entire vulva as well as your bladder, womb and part of your bowel.

If a section of your bowel is removed it will be necessary to create a hole in your abdomen (a stoma), so the bowel can be passed through the stoma, into collection pouches that you wear next to your body.

This is known as a colostomy.

If your bladder is removed there are a number of options open to you. As with a colostomy, your urine can be passed out of your body into a pouch via a stoma, known as an ileal conduit urinary diversion. Or it may be possible to create a “new bladder” by removing a section of your bowel and using it to create a pouch to store urine in.

The section on complications of bladder cancer has more detailed information on the treatment options available to people who have had their bladder surgically removed.

Reconstruction

If only a small amount of tissue has been removed, the skin of the vulva can 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 any wound in your vulva.

Another option is to have a skin flap, where an area of skin near the vulva is used to create a flap and cover the wound.

Radiotherapy

Radiotherapy involves using high-energy radiation, usually X-rays, to destroy cancerous cells.

Radiotherapy can be delivered as:

• internal radiotherapy – where a radioactive implant is placed directly into cancerous tissue
• external radiotherapy – where a machine beams the radioactive waves onto the section of the body that contains the cancer, from the outside 

Radiotherapy can be used in three ways for vulval cancer. It can be given:

• before surgery to try and shrink a large cancer – to help make the operation possible without removing nearby organs
• after surgery to destroy any cancerous cells that may be left – for example for cases where cancer cells have spread to the lymph nodes in the groins
• to relieve symptoms in cases where a complete cure is not possible – this is known as palliative radiotherapy

In some cases radiotherapy can be used, or combined with chemotherapy, as an alternative to surgery. This may be possible if it looks like surgery will cause extensive damage to your bladder or bowel and you are unwilling to have a stoma or new bladder.

Internal radiotherapy can be given in different ways for vulval cancer and they can vary between specialist centres. If internal radiotherapy is recommended for you, your MDT will be able to provide more information on what will be involved.

External radiotherapy is normally given in daily sessions, five days a week, with each session lasting around 10-15 minutes. Most people require four to five weeks of sessions.

While the radiation is effective in killing cancerous cells, it can also damage healthy tissues leading to a number of side effects, such as:

• sore skin around the vulva area
• diarrhoea 
• feeling tired all the time
• loss of pubic hair
• swelling of the vulva
• narrowing of your vagina, which can make sex difficult
• inflammation of your bladder (cystitis)

In younger women external radiotherapy can sometimes trigger an early menopause. This means they will no longer be able to have any children.

Read more about radiotherapy.

Chemotherapy

Chemotherapy is usually used in combination with radiotherapy or to control symptoms when a cure is not possible. It is also used as an alternative to surgery that would require a colostomy or ileal conduit urinary diversion (see above).

Chemotherapy uses medication which destroys cancer cells. It is usually given by injection.

The medicines used in chemotherapy can sometimes damage healthy tissue as well as the cancerous tissue. The side effects are common and include:

• feeling sick
• being sick
• hair loss
• sore mouth
• mouth ulcers 
• tiredness

These side effects should stop once treatment has finished. Chemotherapy can also weaken your immune system, making you more vulnerable to infection.

It’s important to report any symptoms of a potential infection, such as a high temperature, persistent cough or reddening of the skin to your multidisciplinary team. You should also avoid close contact with people known to have an infection.

Read more about chemotherapy.