Cancer occurs when the cells in a certain area of your body divide and multiply too rapidly. This produces a lump of tissue known as a tumour.

The exact reason why this happens in cases of vulval cancer is unknown, but certain things can increase your chances of developing the condition.

These include:

• increasing age
• vulval intraepithelial neoplasia (VIN)
• human papilloma virus (HPV) infection
• skin conditions that can affect the vulva, such as lichen sclerosus
• smoking

These factors are described in more detail below.

Increasing age

The risk of developing vulval cancer increases as you get older. Most cases develop in women aged 65 or over, although very occasionally women under 50 can be affected.

Vulval intraepithelial neoplasia (VIN)

Vulval intraepithelial neoplasia (VIN) is a pre-cancerous condition. This means there are changes to certain cells in the vulva that aren't cancerous, but could potentially turn into cancer at a later date. This is a gradual process that usually takes well over 10 years.

In some cases, the abnormal cells may go away by themselves. However, because of the risk of cancer, treatment to remove the affected cells is often recommended.

Symptoms of VIN are similar to those of vulval cancer, and include persistent itchiness of the vulva and raised discoloured patches. See your GP if you have these symptoms.

There are two types of VIN:

• usual or undifferentiated VIN – this usually affects women under 50 and is thought to be caused by an HPV infection
• differentiated VIN (dVIN) – this is a rarer type, usually affecting women over 60, associated with skin conditions that affect the vulva

You can read more about HPV and the skin conditions associated with vulval cancer below.

Human papilloma virus (HPV)

Human papilloma virus (HPV) is the name given to a group of viruses that affect the skin and the moist membranes that line the body, such as those in the cervix, anus, mouth and throat. It's spread during sex, including anal and oral sex.

There are many different types of HPV, and most people are infected with the virus at some time during their lives. In most cases, the virus goes away without causing any harm and doesn't lead to further problems.

However, HPV is present in at least 40% of women with vulval cancer, which suggests it may increase your risk of developing the condition. HPV is known to cause changes in the cells of the cervix, which can lead to cervical cancer. It's thought the virus could have a similar effect on the cells of the vulva, which is known as VIN.

Skin conditions

Several skin conditions can affect the vulva. In a small number of cases these are associated with an increased risk of vulval cancer.

Two of the main conditions associated with vulval cancer are lichen sclerosus and lichen planus. Both of these conditions cause the vulva to become itchy, sore and discoloured.

It's estimated that less than 5% of women who develop one of these conditions will go on to develop vulval cancer. It's not clear whether treating these conditions reduces this risk.

Smoking

Smoking increases your risk of developing VIN and vulval cancer. This may be because smoking makes the immune system less effective, and less able to clear the HPV virus from your body and more vulnerable to the effects of the virus.

See your GP if you notice any changes in the normal appearance of your vulva.

Your GP will ask you about your symptoms, look at your medical history, and examine your vulva to look 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, it may help to let your GP surgery know in advance of your appointment.

Referral to a gynaecologist

If your GP feels some further tests are necessary, they will refer you to a hospital specialist called a gynaecologist. A gynaecologist is a specialist in treating conditions of the female reproductive system.

The National Institute for Health and Care Excellence (NICE) recommends that GPs consider referring a woman who has an unexplained vulval lump or ulcer, or unexplained bleeding.

The gynaecologist will ask about your symptoms and examine your vulva again, and they may recommend a test called a biopsy to determine whether you do have cancer. 

Biopsy

A biopsy is where a small sample of tissue is removed so it can be examined under a microscope to see if the cells are cancerous.

This is often done after a local anaesthetic has been given to numb the area, which means the procedure shouldn't be painful and you can go home the same day. Occasionally, it may be done under general anaesthetic (where you're asleep), which may require an overnight stay in hospital.

Your doctor may put a few stitches in the area where the biopsy was taken from. You may have slight bleeding and soreness for a few days afterwards.

Your doctor will usually see you 7 to 10 days later to discuss the results with you.

Further tests

If the results of the biopsy show cancer, you may need further tests to assess how widespread it is.

These may include:

• a colposcopy – a procedure where a microscope is used to check for abnormal cells in the vagina
• a cystoscopy – an examination of the inside of the bladder using a thin, hollow tube inserted into the bladder
• a proctoscopy – an examination of the inside of the rectum
• biopsies of the lymph nodes near your vulva to check whether cancer has spread through your lymphatic system
• a computerised tomography (CT) scan or magnetic resonance imaging (MRI) scan – scans to check for signs of cancer in your lymph nodes or other organs
• an X-ray to check that cancer has not spread to your lungs

The results of these tests will allow your doctor to "stage" your cancer. This means using a number system to indicate how far the cancer has spread.

Staging

Vulval cancers are staged using a number from 1 to 4. The lower the stage, the less the cancer has spread and the greater the chance of treatment being successful.

The staging system for vulval cancer is:

• Stage 1 – the cancer is confined to the vulva
• Stage 2 – the cancer has spread to other nearby parts of the body, such as the lower vagina, anus or lower urethra (the tube urine passes through out of the body), but the lymph nodes are unaffected
• Stage 3 – the cancer has spread into nearby lymph nodes
• Stage 4 – the cancer has spread to other parts of the body, including more distant lymph nodes

Stage 1 and 2 vulval cancers are generally regarded as early-stage cancers with a relatively good chance of being treated successfully. Stage 3 and 4 cancers are usually regarded as advanced-stage cancers and a complete cure for these types of cancers may not always be possible.

Treatment for vulval cancer depends on factors such as how far the cancer has spread, your general health, and personal wishes.

The main options are surgery, radiotherapy and chemotherapy. Many women with vulval cancer have a combination of these treatments.

If your cancer is at an early stage, it's often possible to get rid of it completely. However, this may not be possible if the cancer has spread.

Even after successful treatment, there is a chance the cancer will return at some point later on, so you'll need regular follow-up appointments to check for this.

Your treatment plan

Most hospitals use multidisciplinary teams (MDTs) to treat vulval cancer. MDTs are teams of specialists that work together to make decisions about the best way to proceed with your treatment.

Members of your MDT will probably include a specialist surgeon, a specialist in the non-surgical treatment of cancer (clinical oncologist) and a specialist cancer nurse.

Deciding which 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 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:

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

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 be many weeks or months before you start to feel better.

You surgeon will talk to you about the possible risks associated with the type of procedure you're having. Possible risks include infection, bleeding, blood clots, altered sensation in your vulva, and problems having sex.

Assessing and removing groin lymph nodes

An additional operation may also be required to assess whether the cancerous cells have spread into one or more lymph nodes in your groin, and remove these if they're found to contain cancer. 

Sentinel node biopsy

If the cancer has spread to nearby lymph nodes, it's sometimes possible to only 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

In some cases, some or all of the nodes in your groin may need to be surgically removed. This is called a groin or inguinofemoral lymphadenectomy. Further treatment with radiotherapy may also be recommended.

Removing cancerous lymph nodes reduces the risk of the cancer returning, but it can make you more vulnerable to infection and cause swelling in your legs from a build-up of lymphatic fluid (lymphoedema).

Pelvic exenteration

In cases of advanced vulval cancer or where 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. This is a major operation and isn't carried out very often these days.

If a section of your bowel is removed, it will be necessary for your surgeon to divert your bowel through an opening made in your tummy (a stoma). Stools then pass along this piece of bowel and into a bag you wear over the stoma. This is known as a colostomy.

If your bladder is removed, urine can be passed out of your body into a pouch via a stoma. This is known as a urostomy. Alternatively, 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.

Reconstructive surgery

If only a small amount of tissue has been removed during surgery, the skin of the vulva can often be neatly stitched together.

Otherwise, it may be necessary to reconstruct the vulva using a skin graft, where a piece of skin is taken from your thigh or tummy and moved to the 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.

These reconstructive procedures are usually carried out at the same time as the operation to remove the cancer.

Radiotherapy

Radiotherapy involves using high-energy radiation to destroy cancerous cells. There are several ways it can be used to treat vulval cancer:

• before surgery to try to shrink a large cancer – this is 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 groin
• as an alternative to surgery, if you're not well enough to have an operation
• to relieve symptoms in cases where a complete cure is not possible – this is known as palliative radiotherapy

In most cases, you'll have external radiotherapy, where a machine directs beams of radiation on to the section of the body that contains the cancer.

This is normally given in daily sessions, 5 days a week, with each session lasting a few minutes. The whole course of treatment will usually last a few weeks.

Side effects

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

• sore skin around the vulva area
• diarrhoea 
• feeling tired all the time
• loss of pubic hair, which may be permanent
• 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.

Chemotherapy

Chemotherapy is where medication is used to kill cancer cells. It's usually given by injection.

It's usually used if vulval cancer comes back or to control symptoms when a cure is not possible. Sometimes it may be combined with radiotherapy.

Side effects

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

• tiredness
• feeling and being sick
• hair thinning or hair loss
• sore mouth and mouth ulcers 
• an increased risk of infections – tell your care team if you develop any symptoms of an infection, and try to avoid close contact with people known to have an infection

These side effects should pass once treatment has finished.

Emotional support

The emotional impact of living with vulval cancer can be significant. Many people report experiencing a kind of rollercoaster 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.

Some people experience feelings of depression. If you think you may be depressed, contact your GP or care team for advice. There are a range of treatments that can help.

You may also find it useful to contact one of the main cancer charities, such as:

• Macmillan Cancer Support – the helpline is available on 0808 808 00 00, Monday to Friday, 9am to 8pm
• Cancer Research UK – a cancer nurse helpline is available on 0808 800 4040, Monday to Friday, 9am to 5pm