Cancer of the vulva (vulval cancer) is an uncommon cancer. It usually affects women aged over 60 years, although it can also affect younger women. The most common symptoms are a persistent itch or pain in the vulval area. Some vulval cancers start as a sore or lump in the vulva. Vulval cancer is usually treated by an operation to remove the cancer. Most vulval cancers can be cured. The outlook is good in women who have small cancers that have not spread.
What is the vulva?
The outer (external) sex organs of a woman are known as the vulva. The vulva is made up of two pairs of 'lips'. The outer pair, called the labia majora, is covered in pubic hair. The inner pair is called the labia minora, which are thinner and more delicate.
There are two openings between these lips. One is the vagina which leads to the womb (uterus). The other is the urethra, which is the short tube that carries urine from the bladder. At the front of the vulva is the small organ called the clitoris.
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 may 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 obtain reliable information on treatment options and outlook.
What is vulval cancer?
Cancer of the vulva (vulval cancer) can occur on any part of your vulva. It is very rare. It most commonly develops on the inner edges of your labia majora and your labia minora. It can also sometimes affect your clitoris or Bartholin's glands (small glands on each side of the vagina). It can also occasionally start on the skin between your vulva and your anus (your perineum).
Most vulval cancers are squamous cell cancers. This means they have developed from the skin cells in the outer layer of your vulva. Around 4 in 100 cases of vulval cancers are due to a melanoma which develops from cells in your 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 What Causes Cancer? for more details.
Cancer of the vulva (vulval cancer) is an uncommon cancer. There are just around 1,200 new cases each year in the UK. It usually affects women over the age of 60, but is most common in those who are 85 or over. The numbers of younger women with vulval cancer have increased, but are still small.
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.
- Increasing age. Most cases develop in people over the age of 60.
- A condition called vulval intraepithelial neoplasia (VIN) 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. See separate leaflet called Vulval Intraepithelial Neoplasia for more details.
- Human papillomavirus (HPV) is an infection which is passed between people during sex. Some types of HPV, including types 16 and 18 can lead to VIN developing. However, more than half of all vulval cancers are not related to HPV infection.
- Lichen sclerosus is a condition that causes long-term inflammation of the skin in your vaginal area. Having lichen sclerosus can increase your risk of developing vulval cancer; however, the vast majority of women with lichen sclerosus do not develop vulval cancer in the future.
- 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 cancer of the vulva (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 (nodes) 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 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 if you have VIN or cancer of the vulva (vulval cancer). If you do have vulval cancer, the biopsy will show which type of vulval cancer you have. Results of a biopsy usually take around two weeks.
- One or more of: a CT scan or MRI scan of the tummy (abdomen) and chest, a chest X-ray, blood tests, and sometimes other tests.
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.
- Whether the cancer has spread to other areas of your body (metastasised).
Finding out the stage of your cancer helps doctors to advise on the best treatment options. It also gives a reasonable indication of outlook (prognosis).
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 subtype 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 cancers of the vulva (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 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 for removal of the vulva (called a vulvectomy) 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 glands (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. Generally surgeons will try to perform surgery that will give the best possible result for the least scarring procedure.
In most cases the lymph glands in your groin are usually also removed.
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. Radiotherapy may be advised in addition to surgery. Radiotherapy 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. Alternatively it may be given after an operation. See separate leaflet called Radiotherapy for more details.
Chemotherapy is a treatment of cancer by using anti-cancer drugs which kill cancer cells or stop them from multiplying.
Chemotherapy may be used in three different ways: before an operation, to shrink a large tumour, to give a better chance of the operation being successful; after the operation to give a better chance of cure; if a cancer returns (recurs).
There are many different types of chemotherapy. Which type depends on many factors, including your age, general health and your particular stage of cancer. Your specialist can discuss this with you, if you wish. See separate leaflet called Chemotherapy with Cytotoxic Medicines for more details.
What is the outlook (prognosis)?
The outlook is best in those who are diagnosed when the cancer of the vulva (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.
Dr Louise Newson
Dr Louise Newson
Dr Helen Huins