What Is Vulvar Cancer? What Causes Vulvar Cancer?

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Main Category: Women's Health / Gynecology
Also Included In: Cancer / Oncology
Article Date: 05 Dec 2009 - 6:00 PST

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Vulvar cancer, or vulval cancer is an uncommon cancer of the outer surface area of the female genitalia. The symptoms of vulvar cancer include pain, itching and bleeding of the vulva.

The vulva is made up of the outer part of the vagina, the clitoris, and two pairs of lips surrounding the entrance to the vagina, called the labia majora (the outer pair) and the labia minora (the inner pair).

Primary vulvar cancer begins inside the vulva, and secondary vulvar cancer begins in another part of the body (usually the reproductive system), such as the cervix or ovaries, and then spreads to the vulva.

Types of primary vulvar cancer

There are five main types of vulvar cancer. They are classified according to the type of cells that the cancer begins in:

• Squamous cell carcinoma is the most common type of vulvar cancer, accounting for nine out of ten cases. It develops from the squamous cells, the cells in the outer layer of skin. Squamous cell carcinoma usually grows slowly, over a few years.


• Vulvar melanoma is the second most common type, but is still rare. It develops from the pigment-producing cells of the skin.


• Adenocarcinoma is very rare, developing from the cells that line the glands in the vulva.


• Verrucous carcinoma is a very rare and slow-growing type of vulvar cancer. It looks like a wart.


• Sarcoma is extremely rare, developing in tissue such as muscle or fat under the skin.

Cancer may occur in any part of the vulva but the most common sites are the edges of the inner and outer lips.

Less often, vulvar cancer may affect the clitoris or the two small glands on either side of the vagina (Bartholin's glands).

Most cases of vulvar cancer are in women aged over 60. Early-stage vulvar cancer can occasionally occur in young women in their 20s.

The outlook for vulvar cancer is generally good if the cancer has not spread beyond the vulva. However, the outlook for the melanoma type of vulvar cancer is poor because it is very aggressive and spreads quickly.

The main treatment for vulvar cancer is surgery to remove the cancerous tissue. Radiotherapy and chemotherapy may also be given.

Getting regular gynecologic exams may increase the chance of early detection of vulvar cancer. This results in a better chance of successful treatment.

What are the signs and symptoms of vulvar cancer?

A symptom is something the patient feels and reports, while a sign is something other people, such as the doctor detect. For example, pain may be a symptom while a rash may be a sign.

Recognizing possible signs and symptoms of vulvar cancer may help detect the disease early. This may give a better chance for successful treatment and long-term recovery. Some of the signs and symptoms are:

• A lump, wart-like bumps
• Bleeding that is not from menstruation
• Burning and pain
• Open sore (ulcer)
• Persistent itching
• Skin changes (such as color changes or thickening)
• Tenderness
• Burning pain when passing urine

Some of the above symptoms can also be symptoms of less serious conditions such as thrush. However, it is recommended to seek medical advice.

Vulvar cancer usually does not develop quickly. There is at first a change in cells, as normal cells become abnormal. This is known as the pre-cancerous stage, and does not mean that cancer will necessarily develop. If abnormal cells are found early on, pre-cancerous changes can be treated before they become invasive cancer of the vulva.

What causes vulvar cancer?

Cancer begins with a change in the structure of DNA, which is found in all human cells. DNA provides cells with a basic set of instructions, such as when to grow and when to reproduce. A genetic mutation changes these instructions so that the cells carry on growing and reproducing uncontrollably. This produces a lump of tissue known as a tumor.

Left untreated, cancer can quickly grow and spread to other parts of the body, usually through the lymphatic system. Once the cancer reaches the lymphatic system, it can spread to any other part of the body, including the bones, blood and organs.

The exact cause of vulvar cancer is unclear.

However, evidence suggests that a number of risk factors can increase the likelihood of developing vulvar cancer:

• Human papilloma virus (HPV): It is a family of viruses that affect the skin and the moist membranes that line the body, such as those in the cervix, anus, mouth and throat. There are many different types of HPV, some of which are more high risk than others.
  
  HPV is known to cause changes in the cells of the cervix, which can lead to cervical cancer. It is thought that the virus could have a similar effect on the cells of the vulva. HPV is spread during sexual intercourse, including anal and oral sex. Using unsafe sex practices that increase the risk of HPV infections augment the risk of vulvar cancer.


• Vulvar intraepithelial neoplasia (VIN): This condition can occur in the skin of the vulva, and is linked to infection by HPV. VIN is a form of pre-cancerous changes in the skin of the vulva. Occasionally, the cells change from normal to abnormal, with the potential to become cancerous.
  
  The most common symptom of VIN is a persistent itch, along with thickened, swollen or discolored patches of affected skin. This condition requires treatment or close medical monitoring.


• Vulvar skin conditions: Women who have long-term, non-cancerous skin conditions, such as vulvar lichen sclerosus and vulvar lichen planus, have an increased risk of developing cancer of the vulva. However, this risk is small. The most common symptoms of vulval lichen sclerosis and lichen planus are itchy and sore, discolored skin around the area, which may split, causing stinging and pain. Specially prescribed ointment can control symptoms.


• Smoking: It increases the risk of developing VIN and vulvar cancer, possibly because smoking damages the immune system. Smoking exposes the body to cancer-causing chemicals that may increase the risk of vulvar cancer. Women with a history of genital warts or HPV have an even further increased risk of vulvar cancer if they smoke.


• Age. About 50 percent of women with vulvar cancers are older than 70 when they are diagnosed. This cancer is not limited to older women, however.


• Human immunodeficiency virus (HIV): This virus weakens the immune system, which may increase susceptibility to HPV infections, thereby increasing the risk of vulvar cancer.


• History of melanoma or suspicious moles: A family or personal history of this serious type of skin cancer anywhere on the body, or a family history of unusual moles increases the risk of vulvar melanoma.

How is vulvar cancer diagnosed?

There will be a review of the symptoms and medical history, physical examination to see if there are any lumps or unusual areas of skin. In nine out of every ten women diagnosed with vulvar cancer, their doctor will be able to see a lump or sore area.

If no obvious cause of the symptoms can be found, there will be a referral to a gynecologist for further testing.

Colposcopy and biopsy

Gynecologists use an instrument called a colposcope to look for any abnormalities in the vulva, cervix and vagina. During the examination (colposcopy), a tissue sample will also be taken (biopsy) from the place where the cancer might be.

Anesthetic cream is applied to the vulva and an injection of local anesthetic is given. The tissue sample can then be checked in a laboratory.

There are two types of biopsies:

• Excisional biopsy. If the abnormal area is small a scalpel may be used to make an incision through the skin and remove the entire tumor. Some stitches may be used to sew up the area depending on how much tissue is removed.


• Punch biopsy. If the irregular area is larger, a portion of it may be removed with a small incision or punch biopsy technique. This technique uses a small device to remove a cylindrical piece of skin about one-sixth of an inch (4 millimeters) across. Stitches are not required after punch biopsy.

If the results of the biopsy suggest there is cancer, and there is a risk that the cancer may have spread, further testing may be required.

These tests may include:

• Cystoscopy: This is an examination of the inside of the bladder. It is done to see if the cancer has spread to the bladder. A thin, hollow viewing tube is inserted into the bladder to look for tumors and take a tissue sample.


• Proctoscopy: This is an examination of the inside of the rectum. The doctor either uses a gloved finger on an instrument called a proctoscope to check for abnormalities such as growths on the rectal wall.


• CT scan: This is a computerized X-ray that can take detailed pictures of the inside of the body. It is useful for showing up cancer tumors, and checking whether cancer cells have spread.


• MRI scan: This is a scan that uses radio waves and magnets to produce detailed pictures of the inside of the body. It is used to verify whether cancer has spread.


• X-ray: It may be recommended to make sure that cancer has not spread to the lungs .


• Pelvic examination under anesthesia. With general anesthesia, the doctor can perform a more thorough examination of the pelvis for potential spread of the cancer.

There is currently no screening program in place because vulvar cancer is so rare. However, an examination of the vulva is carried out during routine cervical smear tests. Pre-cancerous conditions can be identified during this examination, and treated early on to prevent vulvar cancer from developing.

Staging of vulvar cancer

Doctors may refer to the tumor using the initials T, N and M. T stands for tumor extent. N is for lymph node spread, and M is for the distant spread of the cancers. Each of these letters has subcategories that further help doctors define the stage of vulvar cancer.

• Stage 0 is an early cancer that has not spread past the skin's surface.


• Stage I signifies a deeper tumor, but one that is less than about three-quarters of an inch (2 centimeters). This cancer has not spread to the lymph nodes or other areas.


• Stage IItumors are those that still have not spread, but are larger than about three-quarters of an inch (2 centimeters).


• Stage III cancer has spread to lymph nodes, nearby tissue or both.


• Stage IVA signifies a cancer that has spread to the lymph nodes on both sides of the pelvis or to the urethra, bladder, rectum or pelvic bone.


• Stage IVB is a cancer that has spread (metastasized) to distant sites in the body (such as your lungs or brain)

What are the treatment options for vulvar cancer?

Treatment options for vulvar cancer depend on the type and stage of cancer and the general health of the patient. Early-stage vulvar cancer can usually be cured with surgery. But if vulvar cancer is at an advanced stage and has spread to other organs, a cure may not be possible. In this situation, radiotherapy and chemotherapy can relieve the symptoms and slow down the spread of cancer.

Surgery

Surgery is the main treatment for cancer of the vulva. The more advanced a vulvar cancer is, the more tissue that may need to be surgically removed. Options include:

• Laser surgery. If the cancer is in the early noninvasive stages, laser surgery is an option. A laser beam is aimed at the layer of the vulva that contains cancer, killing the cancer cells.


• Excision. This procedure is also called a wide local excision or radical excision. It involves cutting out the cancer and about a half-inch (1.3 centimeters) of the normal tissue all the way around it. A margin of normal-looking tissue is cut out to help ensure that all of the cancerous cells have been removed.


• Vulvectomy. Several types of vulvectomy exist:
  
  
  • Skinning vulvectomy removes only the top layer of skin where the cancer is located. Skin can be grafted from another part of the body to cover this area.
  
  
  • Simple vulvectomy involves removing the entire vulva.
  
  These types of vulvectomies are performed in people with noninvasive vulvar cancer.
  
  • Radical vulvectomy is when either the cancer and the deep surrounding tissue (partial vulvectomy) or the cancer and the entire vulva, clitoris and nearby tissue (complete radical vulvectomy) are removed.
    
    Removing large areas of skin and tissue in the vulva may create problems with healing, infection and the ability of the skin grafts to take. The risk of such complications rises with greater tissue removal.
    
    In addition, women who have undergone vulvectomy may have difficulties achieving orgasm. In some cases, this problem may be temporary. Devices called vaginal dilators may help stretch the opening.


• Pelvic exenteration. If the cancer spread is extensive, several organs may be removed: the lower colon, rectum, bladder, cervix, uterus, vagina, ovaries and nearby lymph nodes. If the bladder, rectum or colon is removed, an artificial opening in the body (stoma) will be created to allow waste to be removed in a bag (ostomy).


• Lymph node removal. Vulvar cancer often spreads to the lymph nodes in the groin. These lymph nodes may be removed. This might require tying off a major vein, the saphenous vein. Some doctors will try to avoid closing this vein to prevent additional risk of leg swelling that can occur with this procedure. After the procedure, a suction drain is placed in the incision for several days.
  
  Removing lymph nodes can cause problems with fluid retention, leg swelling and an increased risk of infection of the lymph vessels (lymphangitis), a condition called lymphedema. If this complication develops, compression devices or support stockings may be given to help ease the symptoms.


• Sentinel lymph node biopsy. This procedure may help avoid some of the side effects of lymph node removal. A sentinel node is one that is closest to the area of the tumor that drains fluid from the cancerous area. In this procedure, a blue dye or a radioactive tracer is injected into the tumor area on the day before surgery. The area is then scanned to see where the tumor drains, and this is the side where the surgeon will focus during the next day's surgery.
  
  On the day of surgery, blue dye or radioactive tracer is once again injected, making the sentinel node easy to find and remove. If no cancer cells are found in the sentinel node, no additional surgery is needed. However, if cancerous cells are found additional lymph nodes on that side of the groin need to be removed. If initial testing reveals an already enlarged lymph node, sentinel node biopsy is not usually performed. The surgeon removes and biopsies the swollen node. Sentinel node biopsy is still considered experimental and is not yet widely available.

Radiation therapy

Radiation given from outside the body (external beam radiation) is usually used only to treat the lymph nodes in the groin and pelvis, not the vulva itself. Sometimes it is used in order to shrink a large tumor so that it can be removed with less extensive surgery. Treated skin may look and feel sunburned for six to twelve months.

Chemotherapy

Chemotherapy uses drugs to destroy cancer cells. It can be given through a vein, orally or through the skin (topically). Like radiation, chemotherapy may be used to shrink a large tumor before surgery. Chemotherapy is usually used in combination with radiotherapy or to control symptoms when a cure is not possible (called palliative chemotherapy).

The side effects of chemotherapy may include hair loss, nausea, vomiting and fatigue. Not everyone has side effects, however, and there are now better ways to control some of them. The side effects should stop once treatment has finished.

Reconstructive surgery

Treatment of vulvar cancer often involves removal of some skin from the vulva. The wound or area left behind can usually be closed without grafting skin from another area of the body. However, depending on how widespread the cancer is and how much tissue is removed, reconstructive surgery can be performed.

Vulvar intraepithelial neoplasia

Generally, the tissue containing these precancerous changes is surgically removed before these cells have a chance to turn into cancer. However, some research has found that imiquimod (Aldara), an immune system modulating medication, may reduce the size of these lesions, possibly offering an additional treatment option.

Follow-up

One in ten women experiences recurrence of vulvar cancer. It is important to see a gynecologist at least twice a year after the treatment is finished.

Clinical trials

As vulvar cancer is rare, patients may be asked to take part in a clinical trial. Clinical trials are an important way for health professionals to learn more about the best way to treat specific conditions. Most clinical trials involve comparing a new treatment with an existing treatment to determine whether the new treatment is more or less effective. There is no guarantee of a cure.

Coping and support

Living with cancer is challenging and dealing with the physical effects of vulvar cancer and its treatment can be especially difficult. It may be difficult to come to terms psychologically with the physical changes that are experienced. Although there are no easy answers for coping with vulvar cancer, the following suggestions may help:

• Find ways to make life easier. If there are problems with incontinence or need to change an ostomy bag, for example, sitting in the back of a movie theater, concert hall or meeting room. Sit in an aisle seat on an airplane or train. Allow for breaks when planning long trips, seminars or excursions.
  
  If chronic fatigue is an issue, make rest a priority and take assistance when it is offered. Exercise may help feeling less fatigued and boost spirits. Talk to your doctor about what kind of exercise might be right for you.


• Share your concerns with others. Consider talking to someone you trust about your concerns. This might be a friend, a family member, your doctor, a social worker, a spiritual adviser or a counselor. Talking to other people with vulvar cancer might be helpful. They can explain how they are coping with similar problems.
  
  Join a support group for people with vulvar or gynecologic cancers. They can sometimes be a good resource for practical information about the disease. You may also find strength and encouragement in being with people who are facing challenges similar to yours. After deciding to participate in a group, try it out a few times. If it does not seem useful or comfortable, do not feel forced to continue.


• Do not fear intimacy. A natural reaction to changes in your body may be to avoid intimacy. Although it may not be easy, it is essentially important to discuss these feelings with your partner. For example, a light touch and use of lubricant may help you avoid painful irritation. You may also find it helpful to talk to a therapist, either on your own or together. Touching, holding, hugging and caressing may become far more important to you and your partner. In fact, the closeness you develop may produce greater intimacy than you have ever had.
  
  Intimacy issues may be even more difficult to address for women who are not currently in a committed relationship. They may worry that no one will ever find them attractive or desirable. In that case, the advice and understanding of a medical social worker or other therapist who is knowledgeable about vulvar cancer can be a tremendous help.

How can vulvar cancer be prevented?

• Avoiding risks
  
  Avoiding sexual behaviors that increase risk of sexually transmitted diseases such as HPV and HIV, since both conditions increase risk of vulvar cancer. These behaviors include not having sex at a young age, not having multiple partners, and not having sex with someone who has had multiple partners. Condoms may lessen the risk of HPV transmission, but they cannot fully protect against HPV. Before beginning a sexual relationship with a new partner, it is a good idea for both partners to be tested for sexually transmitted infections.


• HPV vaccination
  
  There is now a vaccine that provides protection against the strains of HPV that are thought to be responsible for most cases of vulvar cancer. HPV vaccination also protects against cervical cancer, which is far more common than vulvar cancer.
  
  Girls should be offered the HPV vaccine as part of their routine childhood immunization program. The vaccine should be given to girls who are 12 to 13 years old, with three doses given over six months.


• Having regular exams
  
  Help prevent invasive vulvar cancer by being aware of the signs and symptoms of vulvar cancer and having regular gynecologic exams to monitor for precancerous changes that may lead to vulvar cancer. When vulvar cancer is detected early, it is highly treatable.


• Quit smoking
  
  By not smoking, the chances of getting vulvar cancer decrease. Smokers are less able to get rid of the HPV infection from the body, which can develop into cancer.

Written by Stephanie Brunner B.A.


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