Cancer of the vulva

Wednesday 1 October, 2014

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On this page: What is vulvar cancer? | What are the symptoms? | How common is it? | What are the risks? | Diagnosis | Staging | Prognosis | Which health professionals will I see? | Treatment | Key points


This section has information about the symptoms, risks, diagnosis and treatment of cancer of the vulva. This type of cancer is also known as vulvar cancer or vulval cancer.

What is vulvar cancer?

Cancer of the vulva can start in any part of the external female sex organs. It most commonly develops in the labia minora, the inner edges of the labia majora and the perineum. Less often, it may involve the clitoris or the Bartholin’s glands.

Types of vulvar cancer
squamous cell carcinoma affects the skin cells of the vulva
vulvar melanoma a type of skin cancer that develops from the cells that give the skin its colour (melanocytes)
adenocarcinoma
  • begins in the glandular cells lining the skin of the vulva 
  • one type is extramammary Paget’s disease, which looks like eczema
verrucous carcinoma slow-growing cancer that looks like a large wart
sarcoma
  • develops from muscle, fat and connective tissue
  • tends to grow faster than other types of vulvar cancer

What are the symptoms?

There are often no obvious symptoms of vulvar cancer. However, you may have one or more of the following:

  • a lump, sore, swelling or wart-like growth on the vulva
  • itching, burning and soreness or pain in the vulva
  • thickened, raised, red, white or dark brown skin patches
  • a mole on the vulva that changes shape or colour
  • blood, pus or other discharge coming from a lesion or sore spot, which may have an offensive or unusual odour or colour (not related to your menstrual period).

Many women don’t examine their vulva, so they don’t know what is normal for them. Some women don’t look because it is difficult to see without a mirror. Others feel uncomfortable examining their vulva. However, if you feel any pain in your genital area or notice any of the symptoms listed above, you should make an appointment with your general practitioner (GP).

How common is it?

Each year, about 300 Australian women are diagnosed with cancer of the vulva. It most commonly affects post-menopausal women. The incidence is highest for women older than 80. However, vulvar cancer can sometimes occur in younger women.

Squamous cell carcinoma is the most common type of vulvar cancer, making up about 9 out of 10 cases. The other types of vulvar cancer are less common.

What are the risks?

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)

Also 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, HPV is a very common virus and most women with HPV don’t develop vulvar cancer.

Other skin conditions

Some skin conditions such as vulvar lichen sclerosus and vulvar lichen planus 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.

Weakened immune system

Women who have had an organ transplant or who have human immunodeficiency virus (HIV) may be at higher risk of developing vulvar cancer because their immune system is not working normally. 

Vulvar cancer is not contagious and it can’t be passed to other people through sexual contact. It is also not caused by an inherited faulty gene so it can’t be passed on to children. 

Diagnosis

If you have any of the symptoms listed above, your first step will be to visit your GP.

See your GP

Your doctor will ask to do a physical examination of your groin and pelvic area. If the area is painful or sensitive, you may be given a local anaesthetic to numb it during the examination.

If you feel embarrassed or scared about having a physical examination, let your doctor know. If you wish, you can also ask for someone else, such as a nurse or a family member, to be present. The doctor may arrange for a vulvoscopy or biopsy, and may also do an internal examination.

Vulvoscopy

During a vulvoscopy, the doctor uses a microscope called a colposcope to view your vulva. It does not enter the body.

You will be asked to lie on your back on an examination table with your knees bent and legs separated. The doctor will apply a vinegar-like liquid to your vulva, which makes it easier to see abnormal cells through the colposcope.

Biopsy

During the vulvoscopy, your doctor will usually take a small tissue sample (biopsy) from the vulva area. A biopsy is the best way to diagnose cancer of the vulva.

The doctor may put a local anaesthetic into the affected area of your vulva to numb it while the biopsy is taken. The biopsy can be done using a small scalpel instrument, which has a circular blade to remove 3–4 mm of tissue.

There shouldn’t be any pain when the sample of tissue is taken from your vulva, but you may feel a little discomfort. Afterwards, your vulva may bleed a little, and sometimes stitches are needed to close up the wound. Ask your doctor how much bleeding to expect after the biopsy, and how to care for the wound and keep it from becoming infected. You may have some soreness, which can be relieved by pain-killers and taking a warm bath.

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, and which type of vulvar cancer it is.

"Why do we have to be ashamed about having vulvar cancer? When you connect with another woman who has had vulvar cancer, you soon find out that there is no need for shame or embarrassment." – Jane
Internal examination

Although the vulva is the outer part of your genitals, the doctor may also ask to do an internal examination to look at your vagina, cervix, bladder and rectum.

Some women with skin conditions such as lichen planus or lichen sclerosus have narrowing of the vagina, so the internal examination will be done under a general anaesthetic.

Pap smear

If you haven’t had one recently, your doctor may do a Pap smear (test) to check the cells inside the vagina and cervix. During this test, you will lie on a table with your knees up and apart. The doctor will gently insert an instrument with smooth, curved sides (speculum) into your vagina. A lubricant is used to guide the speculum. The sides of the speculum spread the vaginal walls apart so the doctor can see your vagina and cervix. An instrument such as a brush or swab is used to remove some cells from the surface of the cervix.

Colposcopy

The doctor may use a colposcope to look inside your vagina. This is like a microscope that helps the doctor to find any abnormality that may be too small to see with the naked eye. The colposcope doesn’t go inside your vagina; the doctor looks through it from the outside. During this examination, the doctor may take a biopsy.

You may also have other tests to view the inside of your bladder and urethra (cystoscopy) or your rectum and anus (proctoscopy). These will be done under a general anaesthetic.

Further tests

Sometimes further tests are needed to determine your general health, the size and position of the cancer, and whether the cancer has spread. These may include:

Blood test

This checks the number of cells in your blood, and how well your kidneys and liver are working.

Chest x-ray

A painless scan of your lungs.

Examination under anaesthetic

The doctor can give you a general anaesthetic so the vulva can be thoroughly examined without any pain.

CT scan

A computerised tomography scan. This scan takes three-dimensional x-rays of the inside of your body.

MRI scan

A magnetic resonance imaging scan. You will lie on the treatment table inside a cylinder that uses a magnetic field to create pictures of your body. Some women feel claustrophobic during this scan. Tell your medical team if you feel anxious, as they may be able to help you relax. 

Before a CT or MRI scan, you may be given an injection or asked to drink a liquid called a contrast to make the images on the computer appear clearer. Some people are allergic to the iodine in the contrast liquid, so tell the doctor if you have any allergies. 

Staging vulvar cancer

Based on the 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. Your doctor may also tell you the grade of the cells. This tells you how quickly the cancer may develop. Knowing the stage and grade of the cancer helps your doctor recommend the most appropriate treatment. Low-grade (grade 1) cancer cells are slow growing and are less likely to spread. High-grade (grade 3) cells look more abnormal and are more likely to grow and spread quickly. 

Stage  Description
Stage 0 carcinoma i-situ Early cancer. Abnormal cells are found only on the surface of the vulval skin.
Stage 1
Cancer is found only in the vulva and/or perineum. The affected area is 2 cm or less in size.
Stage 2
Cancer is found only in the vulva and/or perineum. The affected area is more than 2 cm in size.
Stage 3
Cancer is found in the vulva and/or perineum, and has also spread to the urethra, vagina, anus or lymph nodes.
Stage 4
Cancer has spread beyond the urethra, vagina and anus into the lining of the bladder or bowel. The cancer may also 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. You may wish 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. Instead, your doctor can give you an idea about common issues that affect people with cancer of the vulva.

In most cases, the earlier vulvar cancer is diagnosed, the better the chances of successful treatment. Many women manage the condition for years with regular check-ups with their specialists.

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, general fitness and medical history are all important in assessing your prognosis.

Which health professionals will I see?

Your GP (general practitioner) will probably arrange the first tests to assess your symptoms. You will then be referred to a gynaecological oncologist. You will be cared for by a range of health professionals who specialise in different aspects of your treatment. This is called a multidisciplinary team or MDT.

Health professional Role
gynaecological oncologist a surgeon who specialises in treating gynaecological cancers, such as cancer of the vulva
radiation oncologist
prescribes and coordinates the course of radiotherapy
medical oncologist
prescribes and coordinates the course of chemotherapy
cancer care coordinator
provides support throughout treatment and liaises with other health professionals
nurses
administer treatment and provide support and assistance through all stages of your treatment, including recovery
dietitian recommends the best eating plan to follow when you are in treatment and recovery
sex counsellor
helps you deal with physical or emotional issues affecting your sexuality
social worker
advises you on support services
physiotherapist, occupational therapist
assist you with getting back to normal activities
 counsellor, psychologist  provide emotional support and help manage anxiety and depression

Treatment

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

Surgery is the main treatment for cancer of the vulva. The type of operation you have depends on the stage of the cancer. One of the following types of surgery may be done:

Laser surgery

This operation uses a narrow beam of intense light (laser) as a knife to remove a small tumour. This is not done to treat invasive cancer.

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 nearby lymph nodes in the groin may also be removed (known as lymph node dissection).

Removing part of the vulva (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.

Removing the whole vulva (radical vulvectomy)

The surgeon removes the entire vulva, including the clitoris. Usually, surrounding lymph nodes are also removed.

Removing vulval cancer that has spread (pelvic exenteration)

This operation removes all the affected organs, such as the lower bowel, bladder, uterus or vagina. This operation is no longer commonly done. Your surgeon will only consider this if you are fit enough to make a good recovery. However, it may be done for advanced cancer that has spread beyond the vulva.

The surgeon will aim to remove all of the cancer while preserving as much normal tissue as possible. However, it is essential to remove a margin of healthy tissue around the cancer to reduce the risk of the cancer coming back (recurring) in this area.

It is usually possible to stitch the remaining skin together, but if a large area of skin is removed, 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 onto 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.

Lymph node dissection

The lymph nodes (also called lymph glands) are part of the lymphatic system. Vulvar cancer often spreads first to the lymph nodes in the groin, so these nodes are often removed from one or both sides of the groin. This is called an inguinal lymph node dissection.

The surgeon may perform a sentinel lymph node biopsy. You will have an anaesthetic and an injection of radioactive dye near the site of the cancer. This is done to identify the lymph node most likely to be the first to have cancer spread to it. The dye will flow to this node, and the surgeon will remove it and determine whether it’s necessary to remove more lymph nodes. Your doctor will talk to you about this type of biopsy and the associated risks.

Sometimes removing lymph nodes in the groin can stop or slow the natural flow of lymphatic fluid. When this happens, it can cause the legs to swell. This is known as lymphoedema

What to expect after surgery
  • Pain-killers

    You will have medication to reduce any pain. Some women have an injection into a space around their spinal cord, called an epidural. This numbs the body from the waist down.

  • Catheter

    A tube called a catheter will drain urine and will help keep your wound clean and dry. This will be removed within a few days.

  • Wound care

    Stitches usually dissolve and disappear as the wound heals, otherwise they will be removed within a couple of weeks. The wound will need to be kept clean and dry and the nurses will show you the best way to do this. Some women have a dressing that is changed regularly. The surgical site will be washed regularly (peri toilets). If your vulva is numb, be careful patting it dry because you won’t realise how much pressure you are using. Some women prefer to use a hair dryer on a low heat setting to dry the area.

Recovery after surgery

Your recovery time after the operation will depend on the type of surgery you have. If a small amount of skin is removed, the wound will probably heal quickly. If your lymph nodes are removed or the surgery is more extensive, recovery will take longer. Women with stitches should try to keep their knees together when getting out of bed so the stitches do not tear.

The emotional impact of having cancer and surgery is significant, and you may wonder how it could affect your sexuality. See sexuality and intimacy.

Using the toilet

If the opening to your urethra was removed or affected, you may find that going to the toilet is different. The urine stream might spray in different directions or go to one side. See managing side effects.

Exercise and movement

Start gently moving around as soon as your doctor says it’s okay. If you have skin grafts or flaps, or if you have had a groin lymph node dissection, you may need bed rest for a few days. The nurse or physiotherapist can help you do regular leg and breathing exercises, and you can ask for pain relief before you start moving around. When you return home, you will need to take it easy. You may not be able to lift anything heavy or drive for 6–8 weeks.

Surgical drains

After some operations, women have a surgical drain placed in the wound to draw fluid away from the incision. The drain needs to remain in place until it is not draining too much fluid, so you may go home with it still in place. Community nurses will help you manage the care of the drain at home until it is removed. 

Radiotherapy

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

External radiotherapy (or external beam) is the most common type of radiotherapy for cancer of the vulva. You will lie on a treatment table while a machine, called a linear accelerator, directs radiotherapy at the affected area of the vulva. Treatment is usually given daily over a few weeks – the number of radiotherapy sessions you have will depend on the type and size of the cancer. Each treatment takes about 10–15 minutes.

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, see below. External radiotherapy will not make you radioactive. It is safe for you to be with other people, including children, after your treatment.

 

Linear accelerator

 

Internal radiotherapy

Internal radiotherapy (brachytherapy) delivers 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 a special temporary applicator will be inserted into your vulva. Radioactive sources, such as pellets, are inserted into the applicator to deliver treatment. You can have this treatment as an inpatient in hospital (low-doserate treatment), or receive shorter outpatient treatment sessions (high-dose-rate treatment). Ask your doctor or nurse for details regarding safety precautions for visitors.

Side effects of radiotherapy

You may have some of the following side effects:

Skin redness, soreness and swelling

After radiotherapy the vulva may become sore and swollen. Wash the area with lukewarm water and avoid using perfumed products, lotions or talcum powder.

Hair loss

You may lose your pubic hair. For some women, this can be permanent.

Lymphoedema

Radiation to the groin can increase the risk of swelling in the legs.

Narrowing of the vagina

Radiotherapy can cause your vagina to shorten and narrow, which may make sex uncomfortable or difficult. For information, see managing side effects.

Cystitis

This is inflammation to the bladder lining. Cystitis can make you feel like you want to pass urine frequently and/or give you a burning sensation when you pass urine.

Diarrhoea

Radiotherapy can irritate the bowel and cause you to have loose stools. If this is a problem, let your doctor know. See managing side effects.

Chemotherapy

Chemotherapy uses cytotoxic drugs to kill or slow the growth of cancer cells. Treatment is often given:

  • during the course of radiotherapy, to make the 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.

Chemotherapy may be given as tablets, in a cream applied to the vulva or, more commonly, by injection into a vein (intravenously). Most women 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 need to spend a few days in hospital.

Side effects of chemotherapy

There are many different types of chemotherapy drugs and the side effects vary depending on the drugs you are given. Your medical oncologist or nurse will discuss the likely side effects with you, including how they can be controlled with medication.

Common problems include feeling sick (nausea), tiredness and a reduced resistance to infections. Chemotherapy for vulvar cancer may also increase any skin soreness caused by radiotherapy. Some people find that they are able to continue with their usual activities during treatment, while others find they need to take things more slowly. For more information, call Cancer Council 13 11 20 or see chemotherapy.

Palliative treatment

Palliative treatment helps to improve quality of life by reducing symptoms of cancer when it is no longer possible to cure the disease. It is particularly important for people with advanced cancer, but it’s not just for end of life care and it can be used at different stages of cancer.

Pain relief is often the focus of palliative treatment. This may be managed with radiotherapy, chemotherapy or other medication. However, palliative treatment can also involve the management of other physical and emotional issues.

You can call Cancer Council 13 11 20 for free booklets about palliative care and advanced cancer.

Key points

  • Cancer of the vulva is also known as vulvar or vulval cancer. There are several types, and it can start in any part of the vulva.
  • Symptoms may include a lump, burning, itching, pain, coloured skin patches or a mole that changes in colour.
  • Some factors, such as vulvar intraepithelial neoplasia (VIN), can increase the risk of developing vulvar cancer.
  • Your doctor will confirm the diagnosis with a physical examination of the groin and pelvic area, biopsy and scans.
  • The stage of the cancer describes its size and if it has spread. The grade tells how quickly the cells are growing.
  • The earlier vulvar cancer is diagnosed, the better the chances of successful treatment.
  • You may see a range of health professionals, including a gynaecological oncologist.
  • Surgery is the main treatment for vulvar cancer. The type of operation you have depends on the stage of the cancer. The lymph nodes in the groin may also be removed.
  • Radiotherapy uses x-rays to destroy cancer cells. You may have external radiotherapy or internal radiotherapy (brachytherapy). Side effects vary depending on the type of radiotherapy you have.
  • Chemotherapy uses anticancer drugs to kill cancer cells. Side effects can include nausea and fatigue.
  • Palliative treatment may be given to manage cancer symptoms and improve quality of life. 

Reviewed by: Prof Jonathan Carter, Head Gynaecologic Oncology, Chris O’Brien Lifehouse, Professor of Gynaecological Oncology, University of Sydney, and Head Gynaecologic Oncology, Royal Prince Alfred Hospital, NSW; Ellen Barlow, Gynaecological Oncology Clinical Nurse Consultant, Gynaecological Cancer Centre, The Royal Hospital for Women, NSW; Jason Bonifacio, Practice Manager/ Chief Radiation Therapist, St Vincent’s Clinic, Radiation Oncology Associates and Genesis Cancer Care, NSW; Wendy Cram, Consumer; Kim Hobbs, Social Worker, Gynaecology Oncology, Westmead Hospital, and Chair COSA Social Work Group, NSW; Lyndal Moore, Consumer; Pauline Tanner, Cancer Nurse Coordinator, Gynaecological Cancer, WA Cancer and Palliative Care Network, WA.
Updated: 01 Oct, 2014