Vulval Cancer

What is the vulva?

What is cancer?

Cancer is a disease of the cells in the body. The body is made up from millions of tiny cells. There are many different types of cell in the body, and there are many different types of cancer which arise from different types of cell. What all types of cancer have in common is that the cancer cells are abnormal and multiply 'out of control'.

A malignant tumour is a 'lump' or 'growth' of tissue made up from cancer cells which continue to multiply. Malignant tumours invade into nearby tissues and organs which can cause damage.

Malignant tumours may also spread to other parts of the body. This happens if some cells break off from the first (primary) tumour and are carried in the bloodstream or lymph channels to other parts of the body. These small groups of cells may then multiply to form 'secondary' tumours (metastases) in one or more parts of the body. These secondary tumours may then grow, invade and damage nearby tissues, and spread again.

Some cancers are more serious than others, some are more easily treated than others (particularly if diagnosed at an early stage), some have a better outlook (prognosis) than others.

So, cancer is not just one condition. In each case it is important to know exactly what type of cancer has developed, how large it has become, and whether it has spread. This will enable you to get reliable information on treatment options and outlook.

See separate leaflet called 'Cancer - What are Cancer and Tumours' for further details about cancer in general.

What is vulval cancer?

Vulval cancer can occur on any part of the vulva. It most commonly develops on the inner edges of the labia majora and the labia minora. It can also sometimes affect the clitoris or the Bartholin glands (small glands on each side of the vagina). It can also occasionally start on the perineum (the skin between the vulva and the anus).

Most vulval cancers are squamous cell cancers. This means they have developed from the skin cells in the outer layer of the vulva. Around 4 in 100 cases of vulval cancers are due to a melanoma which develops from cells in the skin that cause pigmentation.

What causes vulval cancer?

A cancerous tumour starts from one abnormal cell. The exact reason why a cell becomes cancerous is unclear. It is thought that something damages or alters certain genes in the cell. This makes the cell abnormal and multiply 'out of control'. (See separate leaflet called 'Cancer - What Causes Cancer' for more details.)

Vulval cancer is an uncommon cancer. There are around 1000 new cases each year in the UK. It usually affects women between the ages of 55 and 75. However, it is becoming more common in younger women.

In many cases, the reason why a vulval cancer develops is not known. However, there are factors which are known to alter the risk of vulval cancer developing. These include:

• Age. Most cases develop in people over the age of 55.
• A condition called VIN (vulval intraepithelial neoplasia) can occur in the skin of the vulva. The most common symptom of VIN is a persistent itch. Areas of skin affected by VIN can look thickened and swollen, with red, white or dark coloured patches. Around one third of vulval cancers develop in women who have VIN.
• Human papilloma virus (HPV). This is an infection which is passed between people during sex. Some types of HPV, including types 16, 18 and 31 can lead to VIN developing. However, more that half of all vulval cancers are not related to HPV infection.
• Lichen sclerosus and lichen planus. These are two conditions that cause long-term inflammation of the skin in the vaginal area. Although almost two-thirds of vulval cancers occur in women who have lichen sclerosus, only between one and two in a hundred women who have lichen sclerosus will develop vulval cancer.
• Genital herpes. Infection with the genital herpes virus type 2 increases the risk of vulval cancer. However, most women who have genital herpes do not develop vulval cancer.
• Smoking. Smoking increases the risk of developing both VIN and vulval cancer.

Note: vulval cancer is not an inherited condition and does not usually run in families.

What are the symptoms of vulval cancer?

The symptoms of vulval cancer can vary between women. They may include:

• A persistent itch.
• Pain or soreness in the vulval area.
• Thickened, raised, red, white or dark patches on the skin of the vulva.
• An open sore or growth that does not improve.
• Burning pain when you pass urine.
• Vaginal discharge or bleeding.
• A lump or swelling in the vulva.
• A mole on the vulva that changes shape or colour.

Note: All these symptoms can be caused by other conditions which are not cancer. If you have any of these symptoms, then you should see your doctor.

Vulval cancer 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.

How is vulval cancer diagnosed and assessed?

Anyone who has an abnormal growth or sore on their vulva will have a thorough examination by their doctor. This may include feeling for any enlarged lymph glands in the groin. You will then be referred to see a specialist in the hospital.

It is likely that further tests in the hospital will be arranged. These may include:

• A biopsy. This is where a small sample of tissue is removed from the affected area of your vulva. The tissue is then looked at under a microscope and can help to show whether you have VIN or vulval cancer. If you do have vulval cancer, the biopsy will show which type of vulval cancer you have. Results of a biopsy can take two weeks.
• Other tests. These may include one or more of: a CT or MRI scan of the abdomen and chest, a chest X-ray, blood tests, and sometimes other tests. (See separate leaflets on 'CT Scan', 'MRI Scan', 'X-ray Tests', for more detail.)

This assessment is called 'staging' of the cancer. The aim of staging is to find out:

• Whether the cancer has spread to local lymph glands (nodes).
• Whether the cancer has spread to other areas of the body (metastasised).

Finding out the stage of the cancer helps doctors to advise on the best treatment options. It also gives a reasonable indication of outlook (prognosis).

See separate leaflet called 'Cancer - Cancer Staging and Grading' for details.

What are the treatment options for vulval cancer?

Treatment options which may be considered include surgery, radiotherapy and chemotherapy. The treatment advised for each case depends on various factors such as the stage of the cancer (how large the cancer is and whether it has spread), the exact sub-type or 'grade' of the cancer, and your general health.

You should have a full discussion with a specialist who knows your case. They will be able to give the pros and cons, likely success rate, possible side-effects, and other details about the various possible treatment options for your type of cancer.

You should also discuss with your specialist the aims of treatment. For example:

• In some cases, the treatment aims to cure the cancer. Some vulval cancers can be cured, particularly if they are treated in the early stages of the disease. (Doctors tend to use the word 'remission' rather than the word 'cured'. Remission means there is no evidence of cancer following treatment. If you are 'in remission', you may be cured. However, in some cases a cancer returns months or years later. This is why doctors are sometimes reluctant to use the word cured.)
• In some cases, the treatment aims to control the cancer. If a cure is not realistic, with treatment it is often possible to limit the growth or spread of the cancer so that it progresses less rapidly. This may keep you free of symptoms for some time.
• In some cases, treatment aims to ease symptoms. For example, if a cancer is advanced then you may require treatments such as painkillers or other treatments to help keep you free of pain or other symptoms. Some treatments may be used to reduce the size of a cancer which may ease symptoms such as pain.

Surgery

Surgery is the main treatment for vulval cancer. The operation performed depends on the size and position of the cancer.

If the cancer is small, then the cancer and a small amount of surrounding normal tissue can be removed. For larger cancers, an operation called a vulvectomy (removal of the vulva) may be performed. This may be a partial vulvectomy in which only part of the vulva is removed.

Alternatively, this may be a radical vulvectomy in which the entire vulva including the inner and outer labia and the clitoris are removed, usually with the surrounding lymph nodes. If a large amount of skin is removed in the operation, then you may need to have a skin graft or skin flaps. Your surgeon will be able to talk to you about this in more detail.

Radiotherapy

Radiotherapy is a treatment which uses high energy beams of radiation which are focused on cancerous tissue. This kills cancer cells, or stops cancer cells from multiplying. (See separate leaflet called 'Radiotherapy' for more details.) Radiotherapy may be advised in addition to surgery which aims to kill any cancerous cells which may have been left behind following an operation.

Radiotherapy is sometimes given before an operation to shrink the cancer so a smaller operation can then be performed.

Chemotherapy

Chemotherapy is a treatment of cancer by using anti-cancer drugs which kill cancer cells or stop them from multiplying. (See separate leaflet called Chemotherapy for more details.)

Chemotherapy may be given after having an operation. This aims to kill any cancer cells that have been left behind following the operation.

What is the prognosis (outlook)?

The outlook is best in those who are diagnosed when the vulval cancer is at an early stage. Surgical removal of a small vulval cancer gives a good chance of cure.

The treatment of cancer is a developing area of medicine. New treatments continue to be developed and the information on outlook above is very general. The specialist who knows your case can give more accurate information about your particular outlook, and how well your type and stage of cancer is likely to respond to treatment.

Further help and information

Gynae C

Tel: 01793 491116 Web: www.gynaec.co.uk
Offers a national confidential helpline for women who have, or have had, any form of gynaecological cancer.

Macmillan Cancer Support

3 Bath Place, Rivington Street, London, EC2A 3JR
Tel: 0808 800 1234 Web: www.macmillan.org.uk
Provide information and support to anyone affected by cancer.

Cancer Research UK

Web: www.cancerhelp.org.uk provides facts about cancer including treatment choices.

Other support groups

See www.patient.co.uk/selfhelp.asp for a list of support groups for cancer patients.