This information has been prepared to help you understand more about cancer of the vulva.
Women may feel understandably shocked and upset when they are told they have vulval cancer, which is a very rare cancer.
Early detection is vital but often women delay seeking help because of a lack of awareness or embarrassment.
We cannot advise you 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.
What is the vulva?
The vulva is a general term referring to the external female genital organs. The main parts of the vulva are the:
- mons pubis (mount of Venus) – the soft, fatty mound of tissue covered with pubic hair, above the labia
- labia – two large, outer lips (the labia majora), which surround two smaller, thinner inner lips (the labia minora)
- clitoris – a highly sensitive organ found where the labia minora join at the top. When stimulated, the clitoris fills with blood and enlarges in size. Stimulation of the clitoris can result in sexual excitement and orgasm or climax.
The opening of the vagina is below the clitoris. There are also small glands near the opening of the vagina, called Bartholin’s glands, that produce mucus to lubricate the vagina.
The skin between the vulva and anus is called the perineum.
What is cancer of the vulva?
Vulval cancer is an abnormal growth of calls that can occur in any part of the vulva. 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.
Types of cancer of the vulva
There are several types of vulval cancer, depending on which type of cells become abnormal:
Squamous cell carcinoma – Most (90%) cancers of the vulva develop from the squamous cells in the vulva. These cells make up the outer layer of the skin of the vulva. Squamous cell carcinoma 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. The type of melanoma that occurs on the vulva is not sun related.
Adenocarcinoma – These are very rare. Adenocarcinoma of the vulva develops from cells that line glands in the vulval 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 – Slow-growing cancer that looks like a large wart.
Sarcoma – 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 vulval cancer?
About 280 Australian women are diagnosed with vulvar cancer each year. It usually affects post-menopausal women aged 55-75, but it can sometimes occur in younger or older women.
How does vulval cancer develop?
The exact cause of vulvar cancer is unknown, but there are some factors known to increase the risk of developing it:
Vulvar intraepithelial neoplasia (VIN) – This is a pre-cancerous condition of the vulva. The skin of the vulva changes and may itch, burn or feel sore. VIN may disappear without treatment, but it can sometimes become cancerous. About one in three women who develop vulvar cancer has VIN.
Human papillomavirus (HPV) – Sometimes known as the wart virus, HPV is a sexually transmitted infection that can cause women to develop VIN. Although having HPV increases the risk of vulvar cancer, most women with HPV don’t develop it.
Other skin conditions – Non-cancerous (benign) skin conditions such as vulvar lichen sclerosus, vulvar lichen planus and extramammary Paget’s disease can cause itching and soreness, and after many years, may develop into cancer.
Smoking – Cigarette smoking increases the risk of developing VIN and cancer of the vulva. 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.
What are the symptoms of vulval cancer?
There are often no obvious symptoms of vulvar cancer. However, you may have one or more of the following symptoms:
- bloody, pussy or smelly vaginal discharge not related to your menstrual period
- 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
- 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.
Many women don’t look at their vulva, so they do not know what is normal for them. Some women don’t look because it is difficult to see. Others feel uncomfortable or think that their vulva is ugly. However, if you feel any pain in your genital area or notice any of the above symptoms, you should schedule a checkup with your GP.
Tests to diagnose cancer of the vulva
If you have symptoms of vulvar cancer, your doctor will do a physical examination. Local anaesthetic may be used to numb the vulva and surrounding area. Your doctor may also do a blood test to check your general health.
You may have some of the following tests:
During a vulvoscopy, the doctor uses a microscope called a colposcope to examine your vulva. You will lie on your back on an examination table with your legs spread. The doctor will apply some fluid to your vulva, which will make it easier to see abnormal cells. The doctor will use the colposcope – and sometimes a handheld magnifying glass – to look at your vulva.
Your doctor will usually take a small tissue sample (biopsy) during the vulvoscopy. A local anaesthetic will be used to numb the area before the biopsy is taken.
A biopsy is the best way to diagnose cancer of the vulva. The doctor will put a local anaesthetic into the suspicious area of your vulva to numb it and remove some skin. Your vulva may bleed a little, but it is usually not a large wound. Tell your doctor if you feel any pain, as you may be able to have more anaesthetic.
The tissue is sent to a laboratory where a specialist called a pathologist examines the cells under a microscope. The pathologist will be able to confirm whether or not the cells are cancerous.
Although the vulva is the outer part of your genitals, the doctor may also do an internal examination to look at the vagina, cervix, rectum and bladder.
If you haven’t had one recently, your doctor may do a Pap test. During this test, the doctor puts an instrument with smooth, curved sides (speculum) into your vagina. The sides gently spread the vaginal walls apart so the doctor can see your vagina and cervix. A tool such as a brush or spatula is used to scrape some cells from the surface of the cervix.
The doctor may use a colposcope to look inside your vagina. During this examination, the doctor may take a biopsy. You may also have a cystoscopy to examine your bladder and urethra, and a proctoscopy to look inside your rectum and anus. These will be done under a general anaesthetic
Tests to determine the extent of the cancer and if it has spread
Sometimes further tests are needed to determine the size and position of the cancer, and whether it has spread:
A blood sample is taken to check the number of cells in your blood, and to see how well your kidneys and liver are working.
A painless x-ray scan of your lungs, heart or abdomen.
The doctor can give you a general anaesthetic so the vulva can be thoroughly examined without any pain.
A CT (computerised tomography) scan uses X-ray beams to create a detailed, three-dimensional image of the body. The CT scanner is a large, circular machine. The patient lies on a table as the scanner rotates. The scan takes approximately 30 to 40 minutes. To make the image clearer, patients may be asked to drink a special liquid that contains a dye. The dye may cause the patient to experience a hot feeling all over for a few minutes. Tell your doctor if you are allergic to iodine or any dye.
MRI, also called nuclear magnetic resonance imaging or NMRI uses radio waves and a powerful magnet to create a cross-sectional image of the body. The image can show the difference between healthy tissue and diseased tissue. The MRI scanner is like a big metal tube. The patient lies on a table that moves in and out of the tube. The scan takes about an hour to complete. The patient may be injected with a dye before the scan to make the image clearer. Some women feel claustrophobic during this scan – your medical team may be able to give you medication to reduce this feeling.
Staging of vulval cancer
Based on test results, your doctor will tell you the stage of the cancer. This is a way to describe its size and whether it has spread beyond its original site:
Stage 0 (carcinoma in-situ) – early cancer. Abnormal cells are only found on the surface of the vulval skin.
Stage I – Cancer is found only in the vulva and/or perineum. The affected area is 2 cm or less in size.
Stage II – Cancer is found only in the vulva and/or perineum. The affected area is more than 2 cm in size.
Stage III – cancer is found in the vulva and/or perineum, and has also spread to nearby tissues such as the urethra, vagina, anus or lymph nodes.
Stage IV – cancer has spread to the upper urethra, bladder or bowel, local bone spread or spread to both groin nodes and any distant spread.
Your doctor may also tell you the grade of the cells. This tells you how quickly the cancer may develop. Low-grade cancer cells are slow growing and less likely to spread. High-grade cells look more abnormal and are more likely to grow and spread quickly. Knowing the stage and grade of the cancer helps your doctor decide on the most appropriate treatment.
Prognosis means the expected outcome of a disease. You will need to discuss your prognosis and treatment options with your doctor, but it is not possible for any doctor to predict the exact course of your disease.
The earlier the vulval cancer is diagnosed the higher the chances of successful treatment and cure.
Test results, the type of vulvar cancer you have, the rate and depth of tumour growth, how well you respond to treatment, and other factors such as age, fitness and medical history are all important in assessing your prognosis.
Which health professionals will I see?
Health professionals who may care for you while you are being treated for vulval cancer include:
- General practitioner (GP) – arranges the first tests to investigate your symptoms
- Gynaecological oncologist – a surgeon with specialist training in cancer of the female reproductive system and its treatment
- Medical oncologist – prescribes and coordinates targeted therapies and chemotherapy.
- Radiation oncologist – prescribed and coordinates radiotherapy.
- Nurses – support you through all stages of your cancer treatment.
- Cancer care coordinator or clinical nurse consultant (CNC) – supports patients and families throughout treatment and liaises with other staff.
- Dietician – recommends an eating plan to follow while you’re in treatment and recovery.
- Social workers, counsellors, physiotherapists and occupational therapists – link you to support service social worker, and help with emotional, physical or practical issues.
Cancer of the vulva usually takes many years to develop but, like other types of cancer, it is easier to treat and cure at an early stage. Treatment may involve surgery, radiotherapy and chemotherapy. You may have one of these treatments or a combination.
Surgery is the main treatment for cancer of the vulva. The type of operation depends on the stage of the cancer. It may be used either alone or in combination with radiotherapy and chemotherapy. The cancer will be removed using one of the following operations:
Wide local excision – In this operation, the surgeon removes the cancerous part of your vulva and about a 1 cm border of healthy tissue around the cancer (called the margin).
Radical local excision – The surgeon cuts 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 – The affected part of the vulva is removed. The surgeon may also take out some healthy tissue around the cancerous tissue (a wide local excision). This may mean that a significant portion of the vulva is removed.
Radical vulvectomy – The surgeon removes the entire vulva, including the clitoris. Usually, surrounding lymph nodes are also removed.
Pelvic exenteration – This operation is done if the cancer has spread beyond the vulva. The surgeon takes out the affected organs, such as the lower bowel, bladder, uterus or vagina.
The surgeon will aim to remove all of the cancer while preserving as much normal tissue as possible. However, it is important that a margin of healthy tissue around the cancer is removed to reduce e risk of the cancer coming back (recurring) in this area. Usually only a small amount of healthy skin is removed and it is possible to stitch the remaining skin together.
If it is necessary to remove a large area of skin, you may need a skin graft or skin flap. To do this, the surgeon may take a thin piece of skin from another part of your body (usually your thigh or abdomen) and stitch it on to the operation site. It may be possible to move flaps of skin in the vulvar area to cover the wound. The graft or flap will be done as part of the same operation.
Radiotherapy uses high-energy x-rays to destroy or kill cancer cells. Whether you have radiotherapy or not will depend on the stage of the cancer, its size, whether it has spread to the lymph nodes and, if so, how many nodes are affected. You can have radiotherapy:
- before surgery to shrink the cancer and make it easier to remove (neo-adjuvant treatment)
- after surgery to get rid of any remaining cancer cells and reduce the risk of the cancer coming back (adjuvant treatment)
- instead of surgery
- to control symptoms of advanced cancer.
External radiotherapy – this is the most common type of radiotherapy for cancer of the vulva. You will lie on a treatment table and a machine will direct radiotherapy at the cancer. Treatment is normally given as a series of 10-15 minute daily treatments. The number of radiotherapy sessions you have will depend on the type and size of the cancer, but the entire course of treatment will usually last a few weeks.
Radiotherapy to the vulva and groin is painless, but it can cause side effects. The side effects you experience depend on the radiotherapy dose and the length of your treatment
Internal radiotherapy (brachytherapy) – is a way of delivering radiotherapy directly to the tumour from the inside of your body. This is a less common treatment for vulvar cancer. You will be given a general anaesthetic, and thin radioactive needles or wires will be inserted into your body on or near the cancerous tissue. Treatment will make you slightly radioactive, so you will be cared for in a single room in hospital for a few days until the wires or needles are removed. Although it will be safe for your family and friends to visit you for short periods, children and pregnant women won’t be allowed to visit to avoid the chance of them being exposed to radiation.
Chemotherapy uses anti-cancer drugs to kill or slow the growth of cancer cells. Treatment is often given:
- during the course of radiotherapy, to make treatment more effective
- to control cancer that has spread to other parts of the body
- as palliative treatment, to relieve the symptoms of the cancer.
Drugs are sometimes given as tablets or, more commonly, by injection into a vein (intravenously). You will have several treatment sessions, followed by a break. Treatment can often be given to you during visits to a hospital or clinic as an outpatient, but sometimes you may spend a few days in hospital.
Palliative treatment helps to improve people’s quality of life by reducing symptoms of cancer without trying to cure the disease. It is particularly important for people with advanced cancer. However, it is not just for end-of-life care and it can be used at different stages of cancer.
Often treatment is concerned with pain relief and stopping the spread of cancer, but it also involves the management of other physical and emotional symptoms. Treatment may include radiotherapy, chemotherapy or other medication.
After your treatment is over, you will need regular checkups with your doctor or your cancer treatment centre. Blood tests will be done to check your general health and your blood count. Regular checkups can help find a recurrence early, and this gives you the best chance of getting the disease under control.
Checkups will continue for several years but will become less frequent if you have no further problems.
Between follow-up appointments, let your doctor know immediately of any health problems.