Understanding Vulvar Cancer

This information has been prepared to help you understand more about vulvar cancer. The information is an introduction to the diagnosis, treatment and effects of vulvar cancer but is by no means exhaustive. We cannot advise about the best treatment for you. You need to discuss this with your doctors. However, we hope this information will answer some of your questions and help you think about the questions you want to ask your doctors.

• The vulvar
• What is vulvar cancer?
• How common is it?
• Risk factors
• Symptoms
• Prevention and detection
• Diagnosis
• Further information

The vulvar

The vulva is the external part of a woman’s sex organs. It consists of soft fatty tissue covered with pubic hair called the Mons Pubis (Mount of Venus), which is above the labia. The labia have two outer larger lips (the labia majora), which surround two inner smaller and thinner lips (the labia minora).

At the top, where the labia minora join, is a highly sensitive organ called the clitoris. When stimulated, the clitoris fills with blood and enlarges in size. Stimulation of the clitoris can result in sexual excitement and orgasm, or climax.

Just below the clitoris is the opening through which women pass urine (the urethra) and below this is the vagina, a tubular passage through which menstrual blood flows, sexual intercourse occurs, and a baby is born.

The area of the skin between the vulva and anus is called the perineum. All these structures are visible from outside the body. Cancer of the vulva may involve any of the external female sex organs. The most common areas for it to develop are the inner edges of the labia majora and the labia minora. Less often, vulvar cancer may also involve the clitoris or the Bartholin’s glands (small glands, one on each side of the vagina). It can also affect the perineum.

What is vulvar cancer?

There are several types of vulvar cancer:

• Squamous cell carcinoma - Most (90%) cancers of the vulva develop from the squamous cells in the vulva. Squamous cell cancers usually grow very slowly over a few years.
  
  
• Vulvar melanoma - Melanomas develop from the melanin, the pigment-producing cells that give the skin its colour. This is the second most common type of vulvar cancer, but is much less common than the squamous cell type. Only about 4% of vulvar cancers are melanoma.
  
  
• Adenocarcinoma - These are very rare. Adenocarcinoma of the vulva develops from cells that line glands in the vulvar skin. Paget’s disease of the vulva is a pre-malignant condition where glandular cells spread out from these glands and across the skin of the vulva.
  
  
• Verrucous carcinoma - This is a rare, very slow-growing type of vulvar cancer, which looks like a large wart.
  
  
• Sarcomas - These are extremely rare. Sarcomas develop from cells in tissue, such as muscle or fat under the skin, and tend to grow more quickly than other types of cancer.

How common is it?

Cancer of the vulva is rare, with around 200 women diagnosed in Australian each year. It usually affects postmenopausal women between the ages of 55 and 75, but can occur in younger or older women and is becoming more common in younger women.

Risk factors

Although the cause of cancer of the vulva remains unknown, there are some known risk factors:

• Vulvar cancer has been linked to certain precancerous conditions. A condition called VIN (vulvar intraepithelial neoplasia) occurs in the skin of the vulva and can develop into vulvar cancer if left untreated. The human papilloma virus (HPV), or wart virus, appears to be associated with VIN. Almost one-third of vulvar cancers develop in women who have VIN. Women who have had multiple sex partners may be more exposed to a variety of HPV, but women who have had only one sex partner can develop VIN. In younger women, a precancerous lesion (area of tissue) is more likely to be associated with HPV, and this increases the risk of vulvar cancer. This risk is increased in women who smoke. Older women who get vulvar cancer usually don’t have a link with HPV.
  
  
• Skin conditions: women who have certain non-cancerous skin conditions for a long time have an increased risk of developing vulvar cancer. These conditions, called vulval lichen sclerosus and vulval lichen planus, affect the skin in the vulvar area. The skin can become inflamed and itchy, and split and crack, causing pain. The vulva may become distorted and change in shape and size. Almost two-thirds of vulvar cancers occur in women who also have lichen sclerosus, but only a small percentage (1-2%) of women with lichen sclerosus will go on to develop vulvar cancer.
  
  
• Smoking: cigarette smoking increases the risk of developing both VIN and vulvar cancer. This may be because smoking can make the immune system work less effectively.
  
  
• Cancer of the vulva, like other cancers, is not infectious and cannot be passed on to other people. An inherited faulty gene does not cause it and so other members of your family are not likely to be at risk of developing it.

However, many women who have these risk factors do not develop vulvar cancer.

Symptoms

The most common symptoms of cancer of the vulva are:

• Itching, burning and soreness of the vulva
• A lump, swelling or wart-like growth
• Thickened, raised, red, white or dark patches on the skin of the vulva
• Bleeding or a blood-stained vaginal discharge
• Burning pain when passing urine
• Pain in the area of the vulva
• A sore or ulcerated area on the vulva
• A mole on the vulva that changes shape or colour

Cancer of the vulva usually takes many years to develop but, as with other cancers, it is easier to treat and cure at an early stage. Any of the above symptoms can be a sign of many conditions other than cancer, but always get your doctor to treat them.

Prevention and detection

Prevention is better than cure, so The Cancer Council Australia and its state members work to raise awareness of women’s cancers and to educate women about how to lower their cancer risk.

You and your circle of friends can greatly reduce your risk of developing cancer by:

• Learning what the risk factors are and where possible taking steps to decrease your risk
• Knowing the early warning signs and monitoring your health
• Leading a healthy lifestyle

No formal screening programs exist to check well women for vulva cancer. See your GP if you experience any of the symptoms above. Your GP will begin by doing an internal examination of your vagina and cervix and by taking a Pap smear to check for early cell changes in the vagina or cervix. If your doctor suspects that you have vulvar cancer he or she will refer you to a specialist for further tests, such as a biopsy where a tissue sample is taken for closer examination.

Diagnosis

Usually you begin by seeing your GP, who will examine you. If there is a chance you have vulvar cancer, you should be referred to a gynaecological oncologist. An oncologist is a doctor who specialises in the treatment of cancer. A gynaecological oncologist diagnoses and treats women with cancer of the reproductive organs, such as cancer of the vulva.

Your doctor may also arrange for you to have a blood test and chest x-ray to check your general health. At the clinic or hospital the gynaecological oncologist will take your medical history and do a full medical examination.

• Vulvoscopy - The medical examination will include an examination of your vulva. A colposcope may be used to identify any abnormal areas. The colposcope is like a small microscope with a bright light that can magnify areas so that the cells can be seen more clearly. The colposcope remains outside the body. A biopsy will be taken.
  
  
• Internal examination - You will also have an internal examination to check your vagina and cervix for any abnormality. A cervical smear test may be done if you haven’t had one recently. Some women with lichen planus or lichen sclerosus can have narrowing of the vagina so they may need to have the smear under a general anaesthetic. The doctor may also examine your back passage (anus) to check for any abnormal lumps.
  
  
• Biopsy - A biopsy is the best way to diagnose cancer of the vulva. Anaesthetic cream is applied to the vulva to numb the area, and an injection of local anaesthetic is given. The doctor takes the biopsy (a small amount of cells) from the abnormal area. A pathologist examines the biopsy under a microscope.

Further information

For further information on cervical cancer, call The Cancer Council Helpline on 13 11 20 or contact your local state or territory office of The Cancer Council (see the contact us page for details)

© 2008 Cancer Council