Cancer is a genetic mutation of the way cells grow in the body. Changes in the DNA cause cells to over produce in an uncontrolled way creating a cell mass, commonly known as a tumour. If left untreated the cells continue to grow and the cancer can spread to other organs and systems in the body.
 

Vulval cancer is relatively rare. It affects a woman’s external sexual organs, most often the inner edges of the two pairs of lips on the vulva, called the labia minora (inner lips) and the labia majora (outer lips). More rarely it can occur on the skin between the lips, the clitoris and the skin between the vulva and the anus (perineum). It mainly affects older women, the majority of cases affect women over 70. There is also a pre cancerous form (VIN) which is more commonly found in women aged between 30 and 50.

There are five main types of vulval cancer:

Squamous cell carcinoma- this affects the cells in the outer layer of skin on the vulva and is the most common type.

Vulval melanoma- is less common, it originates from the cells of the skin which produce pigment.

Adenocarcinoma- is rare, this originates in the cells that line the glands in the vulva.

Verrucous carcinoma- is slow growing and looks like a wart on the skin.

Sarcoma- develops in the muscle or fat under the skin.

Symptoms

Symptoms for other common conditions can be similar to vulval cancer, these include thrush and other infections.

If simple treatment of any symptom does not help and the problem persists it is very important to visit your doctor.

The most common symptoms are:
 

• itching
• persistent discomfort or pain
• soreness of the vulva
• a lump, swelling or ulcer

Less common symptoms include:

• thickened, raised or coloured patches on the skin of the vulva
• vaginal discharge or bleeding
• pain, difficulty or burning when passing urine
• a mole on the vulva which changes shape or colour

Diagnosis

The GP will need to examine the area, especially as the symptoms can often be caused by other conditions which may be easily treated. If this is not the case, further investigation will be needed and a referral to a gynaecologist will be required.

Further investigations may include:

Colposcopy- this is an examination using a small microscope to allow doctors to look at the cervix, vagina and vulva more closely. Usually at the same time a biopsy (small sample of tissue) will be taken from the suspected area for analysis.

If vulval cancer is confirmed the doctors may recommend other tests to help asses the stage of the cancer, including scans and x-rays. 

Causes and risk factors 

Most vulval cancers occur in women over 70 and the exact cause is not always known. However, there are some factors which increase the risk of developing vulval cancer:

VIN- Vulval Intraepithelial Neoplasia is pre cancerous changes in the cells of the vulva, which in some women can develop into cancer. The HPV virus is thought to cause these changes.

HPV- Human Papiloma Virus is a group of viruses that in some women can cause abnormal cell changes. The virus is spread by skin to skin contact during sexual intercourse. HPV is thought to be responsible for around 50 percent of vulval cancers.
 

Chronic skin conditions- such as lichen sclerosus, lichen planus and paget’s disease all carry a very small risk of developing into vulval cancer. Women with these conditions should discuss any recent changes or concerns with their doctor.

Smoking-  increases the risk of developing VIN and vulval cancer.

Melanoma- a small number of vulval cancers develop from the pigment producing cells that determine skin colour.

Treatment

Treatment depends on the stage of the cancer, these stages are graded from 0-4, where 4 is the most advanced. The main treatment is surgery to remove the abnormal cancerous cells.

Surgery is used to remove the cancerous cells, along with a border of healthy tissue to ensure no abnormal cells are left behind. The amount of tissue removed will depend on the stage of the cancer; stage 1 will have spread less than stage 4, which will be more advanced.

Radiotherapy uses controlled radiation to destroy cancer cells that surgery cannot remove. It can be also used to help reduce symptoms if surgery is not possible.

Chemotherapy uses drugs to destroy cancer cells. It may be used together with radiotherapy.

Prevention

Screening

There is no screening programme for vulval cancer. However, when going for regular cervical screening the nurse or doctor should also examine the vulva to check for any symptoms.
 

Self examination

Regular self examination is very important, be aware of what is normal for you and if you find any changes get them checked by a health care professional.

Vaccination

Some strains of the HPV virus are thought to cause around 50 percent of vulval cancers in women. The Department of Health recommends that all 12-13yr old girls are vaccinated against HPV. This does not protect against other sexually transmitted infections nor all types of vulval cancer, so protected sex (use of condoms), regular self examination and cervical screening is still essential.

Stop smoking

Smoking  increases the risk of developing VIN and vulval cancer.

As with all cancer a healthy lifestyle and a balanced diet may help to lower the risk.

Balanced diet- this is a lower fat, high-fibre diet, which includes wholegrains and at least five portions a day of fresh fruit and veg.

Exercise- regular exercise.

Please consult your GP before starting exercise if this is not a regular activity or you have not exercised for some time.

Julie’s story-

 Julie was having regular cervical screening as there had been some changes noted on her screening tests, but in the meantime Julie had begun to get itching in her vagina. This would come and go but when it started it just would not stop and was continuous, painful and difficult to live with.  To begin with she tried to identify whether it was a reaction to any of the products she was using day-to-day - fabric softener, bath products, but none of the changes she made seemed to help.

Eventually when she was being seen at one of her regular checkups she asked if they could see anything that might be causing this itch.  Although he was unsure himself as to what it might be he referred her to a colleague who specialised in vulval and vaginal health problems. A biopsy (a small cell sample) under local anaesthetic was taken and sent for testing.  At the time Julie didn’t know whether it was something she should be concerned about, as an eczema sufferer she was used to having skin problems but had no idea that this one might be much more serious. When the results came back she was told that she might have VIN - vulval intraepithelial neoplasia.  Like Cervical intra-epithelial neoplasia these are changes to the cells of the vagina which may then, if left unchecked or untreated, become cancerous. It was also explained that VIN might be linked to HPV infection and Julie was aware that she had been infected as she had had genital warts about 4 years earlier which had then cleared up.

Following the diagnosis Julie underwent laser surgery to remove the cells on the affected area of her vagina - this was done under an epidural local anaesthetic.  It wasn’t a particularly pleasant experience and she did need to have time off work following the procedure.

However, this was not the end of the story, the itching returned and Julie went straight back to the consultant and asked him to have another look and was much more aware of the exact area that was itching - she then underwent further surgery removing a larger area of cells.  She was really relieved to be through this event, but found the experience of it ‘devastating’.

Her husband was incredibly supportive and understanding throughout the whole process.  Before the surgery through many months of painful itching sex had been impossible and although they were keen to start a family, clearly that had to be put off until the problem was finally resolved.  Following surgery, although neither of them was worried about the cosmetic appearance, the resultant scarring made sex uncomfortable to begin with but with patience they were ‘getting back to a normal physical relationship’.  No counselling was offered and Julie says that although she appreciates that the overstretched resources of the NHS might not be able to provide this - they could perhaps have signposted her to where she might find it.

Eventually Julie spoke to her GP about how she was feeling about the experience and the GP was able to refer her on to a counselling service which has provided her with the support that she needed to deal with her feelings about the disease.  To begin with, although she felt relief at being comfortable at last, the fact that the disease had recurred meant that it was difficult for her not to worry about the future.  She also had feelings of guilt and embarrassment because there was a constant reminder of how she had acquired the virus, and even though this was not through promiscuity it was an awareness of past relationships and negative thoughts about sex, about feeling ‘punished and guilty’ that affected her in the present. 

In dealing with her own feelings about this illness Julie is keen to overcome any embarrassment to tell her story, she knows that she took the same precautions as anyone else and that the HPV virus is present in around 80 percent of sexually active women.  She also knows that not many people are aware that it can cause pre-cancerous changes to the vagina as well as the cervix and wants more women to be aware of the disease and the symptoms so that they can be sure to get checked.  Julie is really keen that there should be less embarrassment and shame around VIN and that women should certainly not be afraid to talk to their doctor or to follow up about the emotional affects.  She says ‘it’s not about scaremongering - it’s just - put it on your check list along with the other things you do to stay healthy’.

If you would like to tell us your story so we can help and inform other women; there is more information available HERE

This podcast was recorded in March 2011

Vulval cancer: Text version.

Hello and welcome to our podcast about vulval cancer.

Vulval cancer is less common than some of the other gynaecological cancers and often receives less attention.  Many people are actually not aware that there is a pre cancerous form called VIN and that women should be informed about this.  Today we are speaking to Professor John Shepherd who is a Consultant Surgeon and gynaecological oncologist at the Royal Marsden Hospital.  He is also an expert advisor on gynaecological cancer to the International Federation of Obstetrics and Gynaecology.

Hi John, thanks for joining us. Could you first explain the difference between vulval intraepithelial neoplasia or VIN and vulval cancer?

Yes, vulval intraepithelial neoplasia, VIN is a pre cancer and by definition a superficial pre cancer occurring within the skin, intraepithelial neoplasia, cancer within the skin, so it is not an invasive cancer and therefore has no potential for spreading or metastasising but has a potential for developing into a cancer if not adequately treated in the first place.

Could you tell us how common is vulval cancer and who is usually affected?

Vulval cancer is rare. Approximately a thousand women will get vulval cancer that is truly invasive each year in the United Kingdom.  Overall, about 7,500 to 8,000 women will get cancers of the female genital tract in total.  So if we look at the numbers, 1 in 500 women probably, will develop over a lifetime, a vulval cancer which is considerably less than women getting all the various gynaecological illnesses.

Does this type of cancer specifically affect certain age groups of women?

Vulval cancer usually affects older women, the over 70 year age group but any women can develop pre cancers and cancer of the vulva if they are particularly at risk. So it does occur in younger women and I have seen vulval cancer as early as the early 20s, in fact the late teens and a number of women in their 20s. So, any woman who has a persistent lesion, ulcer or wart that doesn’t disappear should quite definitely go and have that inspected and biopsied, and the earlier it is picked up the less of a treatment will be necessary.

There is no screening programme for VIN and vulval cancer so what should women be looking out for?

Lesion or wart that is persistent or an ulcer, perhaps even a wart, that has then ulcerated and then started bleeding is really one of the cardinal symptoms and signs.  The persistence of itching that is unexplained and has failed to respond to more conservative measures; creams and emollients should be regarded with suspicion as well.

So, it’s women being aware of something that persists as a long term problem and not just thinking that it’s nothing and going to be seen to be on the safe side?

And those willing to see their general practitioners and if the lesions persist then they should be referred to a gynaecologist.

Are there certain conditions or risk factors that make some women more at risk?

Women who have had an abnormal cervical smear are more at risk because pre cancers of the cervix are associated with pre cancers elsewhere on the female genital tract; the vaginal, the vulva, the perineum, peri anal region, the buttocks.  Smoking is an added risk factor for both cancers and pre cancers of the cervix and of the vulva, and women who have had warts in the past also.  There are a number of women who may be immunosuppressed for some reason, maybe renal transplant patient, patients who’ve had transplants of some form or other or are taking other forms of chemotherapy which might be immunosuppressive.  These women are more at risk because their defence mechanism is compromised.

Diagnosis usually involves having a colposcopy with a small biopsy.  Could you explain a little bit more about that process?

Yes, colposcopy is basically looking at the cervix, in fact to be absolutely accurate colpos  is the Greek for vagina, so colposcopy is looking at, or through the vagina, basically at the cervix with a microscope that magnifies up the cervix and therefore the rest of the lower genital tract between 8 and 40 times.  Similarly it can used to examine the vulva.  One actually could use the lens from the colposcopy just as accurately with monocular vision. It would allow us to look at the skin of the vulva, to use various (stains) of ascetic acid in particular and look at the blood vessel pattern (the micro vascular angio architecture to give it a more precise term) to look at the blood vessel changes and skin pattern and then directly biopsies can be taken- usually under a bit of local anaesthetic and a ‘punch biopsy’ to look and see what the histological  - the microscopic changes are like.

You mentioned, well you explained about the difference between VIN and vulval cancer. What are the different stages? Does one progress into the other?

So, VIN or can be mild, moderate or severe; VIN1, 2, or 3, much the same as CIN, cervical intraepthilelial neoplasia.  So VIN3 is high grade pre cancer and will have a greater risk and chance of going on, if not treated, to become an invasive cancer over a number of years.  Invasive cancer implies that the lesion, the abnormality, has spread through the skin, through the base of the membrane which is a histological layer of cells beneath the superficial flaky top surface, if you like, of the skin.  Once the base of the membrane has been broken through then the rest of the tissue of the vulva (or whatever organ, the cervix, we are looking at) is at risk of being invaded by the cancer and therefore blood vessel spread and lymphatic spread can occur and so the tumour can spread and it can metastasise.  And so to break through the base of the membrane, that is the all important difference between a pre cancer and a cancer.

Can you tell us a little bit more about the treatment options available for women diagnosed with VIN at any stage really?

So the important thing to do is to exclude invasive cancer and that’s why a biopsy is taken, and then a decision can be taken as to the extent of the treatment that is necessary.  The usual treatment for a pre cancer or VIN lesion would be by excising it surgically with a scalpel, a wide excision, and then primarily closing that incision.  If it’s a very wide area that needs excising, occasionally we have to do some grafting or advancing flaps to cover the raw surface area of the excised skin.  Usually a wide excision and then closing the incision is quite satisfactory.

And should a lot of women be reassured by that with the VIN normally that excision, that removing of that area will solve the problem?

Yes, they should be. There is a need for them to be followed up in the future.  I’ve mentioned the association with cervical lesions and it’s important to make sure that they do have regular cervical smears.  If they are a particular risk, that may have to be yearly but otherwise it can be on the screening programme three yearly.  Having had the VIN excised then these patients will also have to be seen again in a follow up colposcopy clinic so that the colposcopy or vulvoscopy can occur as part of the follow up and ultimately that will be on a yearly basis. 

Could you tell us a little bit more about the treatment options if women are diagnosed with vulval cancer?

So vulval cancer used to be treated in quite a radical way with what was called radical vulvectomy and that involved removing all the skin of the vulva and clitoris and removing the lymph nodes from the groins and from the lower pelvis.  Gradually, over the years we realised that was not necessary in the majority of women and especially with small lesions, a wide local excision is quite adequate.  And then depending on the depth of the cancer a decision is taken as to whether the lymph nodes in the groin need to be removed or not.  We now don’t remove as a standard, lymph nodes in the pelvis because we realised that if the lymph nodes are negative in the groin it’s very unlikely that there will be nodes involved higher up.  But with invasive lesions, if there is more than just a superficial invasion then a groin node dissection is carried out.  It’s usually unilateral on the side of the vulva lesion unless it’s a mid line lesion or the tumour is large and extends onto both sides, in which case a bilateral groin node dissection is necessary.  These incisions now have been modified greatly so that they can be made in a much more cosmetically acceptable way so that (a) it heals better and (b) it is medically not disfiguring.

As well as the surgical aspect of that, would women with vulval cancer ever expect to receive chemotherapy or radiotherapy combined?

If there are lymph nodes that are positive then radiotherapy is usually given to that groin and depending on the risk of there being metastases to the other side, it maybe both groins.  If the excision has been close and there are other risk factors on the vulva or the perineum then radiotherapy can be given to that area as well and chemotherapy is usually given with radiotherapy to sensitise the tumour to the radiotherapy.  Chemotherapy on its own would be given if there were more advanced disease or there were distal metastases.  While the cancer usually spreads locally a small number, 5 to 10% can be much more aggressive. They can then spread, either by lymph node spread or distally higher up in the lymph node chains, the pelvis, the peri-aorta region.  Occasionally, but I emphasise a very small number, will metastasise either into the lungs or the liver and under those circumstances chemotherapy would be given to try and control it.

And as you say, in very rare cases.

Yes, yes.

Could you advise on any questions that women could ask their surgeon if they are facing surgery for VIN or vulval cancer?

Well I think that the important thing is to ensure that there’s pre cancer and not cancer. To ensure that a limited wide excision but conservative approach is taken and that the surgeon that is involved is an expert at dealing with that particular problem, not a generalist but a specialist in gynaecological malignancy.  It’s important that vulva pre cancer should be followed up in a colposcopy setting so that continuous follow up can occur.  Some of these colposcopy clinics occur in a specialised vulva clinic.  Vulva clinics are run by gynaecologists with dermatologists.  And I think that’s a very sensible approach because it means that symptoms such as itching can be treated appropriately. 

Invasive cancer does need a more slightly aggressive approach as I implied, but I think again it’s important to be sure that the cancer is being treated by a specialist in a cancer centre and limited surgery is carried out but it has to be enough surgery.  There are risks to having operations. There are risks to operating on the vulva and the groins especially taking lymph nodes out.  There is a risk of lymphodema so one has to try and limit these risks and I think these risks can be lessened in units and centres in departments that are carrying out a lot of these operations. So the questions should be;

“are you a cancer specialist”, “are you operating with other cancer experts”, be they the physiotherapists and the lymphodema experts, be they the medical oncologists, the clinical oncologists, the radiation oncologists that are treating this disease because the vulva is a very sensitive area for surgery and of course for radiotherapy, and that has to be very carefully worked out. 

The counselling is very important with a clinical nurse specialist to ensure that quality of life is not affected and depending on the age of the patient and her wishes, she may wish to continue with intercourse and if that is the case and it usually is, certainly in the younger women. Counselling is very important to ensure that coitus can resume as appropriate.

Could I quickly just ask you where do you think there will be advances in the future and will the HPV vaccine have a big impact?

I think that the advances in the future are with education of women and highlighting these potential problems.  The vulva is an area that women and doctors don’t often either complain about or examine and there may be a reluctance to complain about a symptom and certainly advanced vulval cancers  will have been present for many months if not years; slowly growing and festering, quite often unpleasant, very large warty lesions which can be quite offensive in terms of a foul discharge and it has taken a lot for the woman to sum up courage to go and see the doctor and regrettably there can be a large reluctance on the part of the doctor, understandably, on the doctor’s behalf to examining the vulva unless there are appropriate circumstances.  So regrettably delay is a problem; education and early reporting of problems and lesions either to the GP or a genitor urinary medicine clinic or a general gynaecology clinic or a woman’s clinic.  The earlier the lesion is picked up the better the chance of curing with lesser surgery and hopefully avoiding other treatments such as radiotherapy.

The HPV vaccine is a very interesting question. Whereas the great majority, if not all, cervical cancers are associated with or caused by one of the papilloma viruses, current evidence is that probably only 50% of vulva cancers are actually caused by the high risk papilloma viruses.  Genital warts themselves are associated with the lesser risk papilloma virus and therefore hopefully, especially with the quadruple vaccine, the incidence of genital warts which are benign and of cervical cancer will decrease.  There will be some decrease in the incidence of vulva cancer but not as dramatically, I suspect, as with cervical cancer.  So the vaccine is important. I think that women should avail themselves, young girls should avail themselves of this and we will see some reduction but we will not see the eradication that we hope to see in cervical cancer.

So it’s important for women to still recognise that?  For girls in the future, even if they’ve had that vaccine to still be aware.

Thanks so much for joining us. It’s great to have your expert opinion.

You’re very welcome. Thank you.

Find out the answers to common myths surrounding vulval cancer.

Vulval cancer only affects older women.

It mainly affects older women, the majority of cases are in women over 70. However, there is also a pre cancerous form (VIN) which is more commonly found in women aged between 30 and 50.

Cervical screening will test for any problems ‘down there’.

The cervical screening test only screens for pre-cancerous changes in the cells of the cervix and cervical cancer. However, during cervical screening the practitioner should observe the vulva and for any symptoms. Regular self examination is important- women should look out for persistently itchy areas of skin, lumps, lesions/sore areas or any unusual changes.

If I have VIN that means I will definitely get vulval cancer.

Pre cancerous changes in the cells of the vulva are called VIN- Vulval Intraepithelial Neoplasia, which in some women can go on to develop into vulval cancer. If VIN is diagnosed then the pre-cancerous cells will be removed. It does not mean that you will have vulval cancer.

If I have a pre-existing vulval condition I am at significant risk.

Lichen sclerosus, lichen planus or paget’s disease all carry a very small risk of developing into vulval cancer. Women with these conditions should discuss any recent changes or concerns with their doctor and regularly self examine for any changes.

To find out about our research follow this link:

A study looking at cells and antibodies that could attack the skin in the vulval diseases lichen sclerosus and lichen planus

• The Royal College of Obstetricians and Gynaecologists website has a wide range of information and guidelines on many women’s health topics. This can be found at: http://www.rcog.org.uk/womens-health/patient-information

• Look Good...Feel Better helps women manage the visible side effects of cancer treatment: www.lookgoodfeelbetter.co.uk

• NHS Choices website also has a range of health information. This can be found at: http://www.nhs.uk/Pages/HomePage.aspx