The Vulva
The vulva is the external part of a woman’s sex organs. 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, vulval 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.
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Types of cancer of the vulva
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.
Vulval melanoma
Melanomas develop from the melanin, the pigment-producing cells that give the skin its colour.
This is the second most common type of vulval cancer, but is much less common than the squamous cell type. Only about 4% of vulval 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.
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.
Around 70 women are diagnosed with vulval cancer in NSW 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 young.
What are the causes?
Precancerous conditions
Although the cause of cancer of the vulva remains unknown, it has been linked to certain precancerous conditions.
A condition called VIN (vulvar intraepitheli al 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, also appears to be associated with VIN. Almost one-third of vulval cancers develop in women who have VIN..More than eight out of ten women will be infected with genital HPV at some time in their lives and for most it will clear up on its own.
In younger women, a precancerous lesion (area of tissue) is more likely to be associated with HPV, and this increases the risk of vulval cancer. This risk is increased in women who smoke.. HPV is not usually associated with vulval cancer in older women.
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 vulval 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 vulval 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 vulval cancer.
Smoking
Cigarette smoking increases the risk of developing both VIN and vulval 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.
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 check them.
Diagnosis
Usually you begin by seeing your GP, who will examine you.
If there is a chance you have vulval 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.
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 tests
Even after a diagnosis is made, further tests are often needed to determine the size and position of the cancer, and whether it has spread. This process is called staging.
The results will help you and your doctor decide on the best treatment for you.
The following tests are most often used with cancer of the vulva.
Stages of vulval cancer
The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site.
Knowing the type and stage of the cancer helps the doctors to decide on the most appropriate treatment.
- Stage 1: Cancer is found only in the vulva and/or in the perineum, which is the space between the opening of the rectum and the vagina. The affected area is 2cm or less in size.
- Stage 2: Cancer is found in the vulva and/or the perineum. The affected area is larger than 2cm.
- Stage 3: Cancer is found in the vulva and/or perineum and has spread to nearby tissues such as the lower part of the urethra (the tube through which urine passes), the vagina, the anus (the opening of the rectum) and/or nearby lymph nodes.
- Stage 4: Cancer has spread beyond the urethra, vagina and anus into the lining of the bladder or the bowel, or it may have spread to the lymph nodes in the pelvis or to other parts of the body.
Prognosis
Prognosis means the expected outcome of a disease.
The earlier the vulval cancer is diagnosed the higher the chances of successful treatment and cure.
Many factors affect prognosis, including the stage of the cancer and your general health. Much of this information will not be known until after the surgery and will be discussed with you when the results of your surgery are available.
For more information, talk to your doctor. Only someone who knows your medical history can tell you what to expect and the treatment options that are best for you.
Treatment
Surgery
Surgery is the main treatment for cancer of the vulva. It may be used either alone or in combination with radiotherapy and chemotherapy.
Your doctor will talk to you about the most appropriate type of surgery, depending on the stage of your cancer.
All operations for cancer of the vulva will remove the area of the skin where the cancer is located.
The cancer will be removed using one of the following operations:
Wide local excision takes out the cancer and a border (margin) of healthy cells, ideally at least 1cm, all around the cancer.
Radical local excision takes out the cancer and a larger area of normal tissue all around the cancer. The groin lymph nodes may also be removed (known as lymph node dissection).
Partial vulvectomy removes part of the vulva.
Radical vulvectomy removes the entire vulva, including the clitoris, and usually the surrounding lymph nodes.
Pelvic exenteration is done if the cancer has spread beyond the vulva. The surgeon removes the affected organs (such as the lower bowel, or the bladder and the cervix, uterus and vagina).
Any surgery aims to remove the cancer while preserving as much normal tissue as possible. Usually only a small amount of unaffected skin is removed with the cancer, so it is often possible to stitch the remaining skin neatly together.
However, if it is necessary to remove a large area of skin, you may need a skin graft or skin flaps. To do this, the surgeon may either take a thin piece of skin from another part of the body (usually the thigh or abdomen) and stitch it on to the operation site. It may be possible to move (rotate) flaps of skin in the vulvar area to cover the wound.
Radiotherapy
Radiotherapy uses x-rays to kill cancer cells or injure them so they cannot multiply.
It can be given externally, where a machine directs radiation at the cancer and surrounding tissue, or internally, where radioactive material is put in thin tubes into your body on or near the cancer.
Radiotherapy may be given to the vulva and the lymph glands after surgery to make sure that any remaining cancer cells are destroyed, and to reduce the risk of the cancer coming back.
Whether you have radiotherapy or not will depend on the stage of your cancer, its size, whether it has spread to the lymph glands and, if so, how many are affected.
Sometimes radiotherapy is given before surgery to shrink the cancer and make it easier to remove and limit the extent of surgery. This is known as neoadjuvant radiotherapy. Chemotherapy may be used at the same time as radiotherapy to improve the effectiveness of treatment.
If the cancer is known to have spread to the lymph glands, radiotherapy may be used instead of surgery to treat this area.
In advanced vulval cancer (where the cancer has come back or spread), radiotherapy may be used to shrink a tumour and reduce symptoms to improve quality of life. This is known as palliative radiotherapy.