Vulvar cancer is an uncommon cancer of the outer surface area of the female genitalia.

Most vulvar cancers are squamous cell carcinomas — a type of skin cancer — that develop slowly over years. A small number of vulvar cancers begin as melanoma. Rarely, vulvar cancers develop in the mucus-producing glands on the sides of the vaginal opening. The sexually transmitted infection human papillomavirus (HPV) is believed to play a role in developing this form of vulvar cancer.

Getting regular gynecologic exams may increase your chance of early detection of vulvar cancer, which means a better chance of successful treatment. You may also be able to prevent vulvar cancer by engaging in safe sexual practices, and you may be able to control other risk factors as well.

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Vulva

Recognizing possible signs and symptoms of vulvar cancer may help you detect the disease early, before it reaches a later stage. This may give you a better chance for successful treatment and long-term recovery. If you experience any of the following vulvar signs and symptoms, see your doctor:

• Itching that doesn't go away
• Burning, pain and tenderness
• Bleeding that isn't from menstruation
• Skin changes, such as color changes or thickening
• A lump, wart-like bumps or an open sore (ulcer)

When to see a doctor
Because an early diagnosis of vulvar cancer increases the likelihood of successful treatment, it's important that you see your primary care doctor or gynecologist if you experience irregular bleeding, persistent itching, burning, pain or tenderness in your genital area, or if you notice skin changes or a lump or open sore on your vulvar area.

If you have already been treated for vulvar cancer, be sure to see your doctor for regular follow-up exams to guard against recurrence of the disease.

The exact cause of each type of vulvar cancer isn't known. Vulvar cancers that occur in older women that aren't linked to HPV infection may be related to a mutation or defect in the p53 tumor suppressor gene. This gene plays a role in keeping cells from becoming cancerous. This type of cancer may also be seen in women with lichen sclerosus — a condition that causes the vulvar skin to become thin and itchy.

As many as 30 percent to 50 percent of vulvar cancers have been linked to the sexually transmitted HPV infection. Many times these women have a precancerous skin condition called vulvar intraepithelial neoplasia in more than one area of the vulva before developing cancer.

Vulvar cancer is uncommon, accounting for less than 1 percent of all cancers in American women.

Although the exact cause of vulvar cancer isn't known, certain factors appear to increase your risk of the disease. These factors include:

• Age. About 50 percent of women with vulvar cancers are older than 70 when they're diagnosed.  This cancer isn't limited to older women, however. As many as 15 percent of new cases occur in women younger than 40.
• HPV infection. This sexually transmitted disease is present in most younger women who have vulvar cancer. Having HPV, or using unsafe sex practices that put you at greater risk of HPV infection, increases your risk of vulvar cancer.
• Smoking. Smoking exposes you to cancer-causing chemicals that may increase your 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.
• Human immunodeficiency virus (HIV). This virus weakens the immune system, which may make you more susceptible to HPV infections, thereby increasing your risk of vulvar cancer.
• Vulvar intraepithelial neoplasia. Though most cases of this precancerous condition won't turn into vulvar cancer, the condition does increase your risk of vulvar cancer and should be monitored by your doctor.
• Lichen sclerosus. About 4 percent of women with this condition, which causes the vulva to become thin and itchy, later develop vulvar cancer.
• A history of melanoma or suspicious moles. If you have a family or personal history of this serious type of skin cancer anywhere on your body, or if you have a personal or family history of unusual moles, you're at increased risk of a vulvar melanoma.

Your first appointment will be with either your primary care physician or a gynecologist. If your doctor or gynecologist suspects or diagnoses cancer, you'll likely be referred to an oncologist who specializes in gynecologic cancers.

Because appointments can be brief, and it can be difficult to remember everything you wanted to discuss, it's a good idea to be well prepared for your appointment. Here are some suggestions for preparing for your appointment, and what you can expect from your doctor.

What you can do

• Write down any symptoms you're experiencing. Include all of your symptoms, even if you don't think they're related.
• Bring a list of any medications or vitamin supplements you take. Write down dosages and how often you take them.
• Bring a family member or close friend with you, if possible. You may be given a lot of information at your visit, and it can be difficult to remember everything. If someone else is with you, they may remember details that you missed or forgot. They may also think to ask important questions you might not have considered.
• Bring a notebook or notepad with you. That way you can write down important information, such as treatment options.
• Prepare a list of questions to ask your doctor. Knowing ahead of time what you want to ask your doctor can help you make the most of your limited time together. List your most important questions first, in case time runs out.

For vulvar cancer, some basic questions to ask include:

• What kinds of tests will I need?
• Do I need to do anything to prepare for these tests?
• Other than vulvar cancer, are there any other possible causes for these symptoms?
• What type of vulvar cancer do I have?
• What stage is my cancer?
• What types of surgical options are available to me?
• What kind of success rates does each type of surgery have?
• What are the drawbacks to each type of surgery?
• Will I need to wear an ostomy bag?
• What about radiation or chemotherapy? Are those options available to me?
• What kind of success rates do those therapies have?
• What types of side effects does each treatment have?
• How will these treatments affect my sexuality?
• Will I be able to have children after treatment?
• How should I prepare for treatment?
• Which course of action do you recommend?
• What are the odds of recurrence?
• What is my prognosis?

And, if your doctor says something that's not clear, don't hesitate to ask him or her questions until you understand completely.

What to expect from your doctor
Your doctor will likely have a number of questions for you. If you're ready to answer them, it may save time for the rest of your appointment. Some potential questions your doctor might ask include:

• When did you first notice these symptoms?
• How often do you experience these symptoms?
• How severe are your symptoms?
• Does anything improve your symptoms?
• Does anything make your symptoms worse?
• Do you have a family history of skin cancer or atypical moles?

To check for vulvar cancer, your doctor will first conduct a physical examination, including a pelvic exam. If your doctor finds any irregularities, you'll likely need further testing.

Biopsy
Because signs and symptoms of vulvar cancer can also be caused by a noncancerous (benign) condition, your doctor will need to confirm a diagnosis by removing a small sample of tissue (biopsy) from the irregular area for analysis under a microscope. By examining this tissue, a doctor can usually tell if your condition is benign or cancerous.

To select the best tissue to sample, your doctor may swab a blue dye across your vulva. This dye will react with certain diseased areas, including those affected by a precancerous condition or by vulvar cancer, causing them to turn blue.

Your doctor might also use a special lighted microscope called a colposcope. The colposcope magnifies the surface, helping your doctor identify areas of abnormal cell growth that can't be seen by the naked eye. Your doctor may also swab the area with a weak acetic acid solution (similar to vinegar), which can cause areas affected by a precancerous condition or by vulvar cancer to turn white, making them even more visible.

Once your doctor determines which area to biopsy, the area will be numbed with a local anesthetic. There are two types of biopsies:

• Excisional biopsy. If the abnormal area is small, your doctor may use a scalpel to make an incision through your skin and remove the entire tumor. Your doctor will use a local anesthetic to numb the area and may use stitches to sew up the area depending on how much tissue is removed.
• Punch biopsy. If the irregular area is larger, your doctor may remove a portion of it with a small incision or punch biopsy technique. This technique uses a small cookie-cutter-like device to remove a cylindrical piece of skin about one-sixth of an inch (4 millimeters) across. Stitches aren't required after punch biopsy.

Staging tests
Staging tests help determine the size and location of your cancer and whether it has spread. They also help your doctor determine the best treatment for you. To gather this information, your doctor may use the following tests:

• Cystoscopy. Using a lighted tube, your doctor examines the inside surface of your bladder. Later stages of vulvar cancer may spread to this area. If your doctor finds irregularities, he or she will remove a sample for biopsy. You may need local or general anesthesia depending on how large a sample is needed.
• Pelvic examination under anesthesia. With general anesthesia, your doctor can do a more thorough examination of your pelvis for potential spread of the cancer.

Imaging tests also can help determine if your cancer has spread. These tests may include:

• Chest X-ray. This X-ray of your chest will determine whether the cancer has spread to your lungs.

• Computerized tomography. Computerized tomography — also called CT or CT scan — is an X-ray technique that produces more-detailed images of your internal organs than do conventional X-ray exams. This test can take as little as a few seconds in newer machines. A CT scan can help your doctor see if cancer has spread to your liver or other organs.

  Some CT scans may require you to ingest a contrast medium or have a contrast medium administered through intravenous injection before the scan. A contrast medium blocks X-rays and appears white on images, which can help emphasize structures in your body. There's a slight risk of allergic reaction when using an intravenous contrast medium. Let your doctor know if you've ever had a reaction to contrast medium in the past.

  CT scans can give your doctor more accurate information about the position and size of the tumor and can reveal swollen lymph nodes that may contain cancer.

• Magnetic resonance imaging (MRI). This test uses a magnetic field and radio waves to create cross-sectional 3-D images of your body. Because of the strong magnet used for this test, you'll be asked to remove any metal jewelry, glasses, and items of clothing with metal clasps or buttons before the start of the test. MRI scans can take up to an hour, the space is confined, and during the test you'll hear a loud, thumping noise. If you're afraid of enclosed spaces, ask your doctor if you might be more comfortable with a light sedative. If the noise of the test bothers you, most MRI centers have headphones so that you can listen to music. MRI scans can provide a wealth of information in one test, with the potential to spot everything from an enlarged lymph node in the pelvis to a distant spread of the cancer to places such as the brain or spinal column.
• Positron emission tomography (PET). Unlike other scanning techniques, a PET scan doesn't produce clear structural images of organs. Instead, it shows images containing areas of more or less intense color to provide information about chemical activity within certain organs and tissues. Tumors often use more energy than healthy tissue does and may absorb more of a radioactive tracer, which allows the tumors to appear on the scan. This test is helpful in determining whether your cancer has spread to your lymph nodes or elsewhere in your body. Some centers may offer a CT-PET scan combination.

Results of staging tests
Your doctor may refer to your 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 your vulvar cancer.

• Stage 0 is an early cancer that hasn't spread past your 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 hasn't spread to your lymph nodes or other areas.
• Stage II tumors are those that still haven't 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 your pelvis or to the urethra, bladder, rectum or pelvic bone.
• Stage IVB is a cancer that has spread (metastasized) to distant sites in your body, such as your lungs or brain.

Treatment options for vulvar cancer depend on the type and stage of cancer and include surgical removal of the tumor, radiation therapy, chemotherapy or a combination of these. Be sure to discuss all of your options with your doctor and weigh the benefits and the risks of each treatment. You may also want to get a second opinion before starting treatment, and in some cases, your insurance company may require it.

Surgery
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. Your doctor aims a laser beam at the layer of your vulva that contains cancer, killing the cancer cells.
• Excision. This procedure, which may also be called a wide local excision or radical excision, involves cutting out the cancer and about a half-inch (1.3 centimeters) of the normal tissue all the way around it. Cutting out what doctors refer to as a margin of normal-looking tissue helps ensure that all of the cancerous cells have been removed.

• Vulvectomy. Several types of vulvectomy exist. A skinning vulvectomy removes only the top layer of skin where the cancer is. Your doctor may graft skin from another part of your body to cover this area. A simple vulvectomy involves removing the entire vulva. These types of vulvectomies are performed in people with noninvasive vulvar cancer. In a radical vulvectomy, your doctor removes either the cancer and the deep surrounding tissue (partial vulvectomy) or the cancer and the entire vulva, clitoris and nearby tissue (complete radical vulvectomy).

  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.

  Additionally, women who've undergone vulvectomy may have difficulties achieving orgasm. In some cases, this problem may be temporary. Scar tissue may narrow the vaginal opening, making sexual intercourse uncomfortable or even painful. Devices called vaginal dilators may help stretch the opening, or your surgeon might suggest skin grafts to widen the vaginal opening.

• Pelvic exenteration. If the cancer spread is extensive, your doctor may remove any or all of these organs: the lower colon, rectum, bladder, cervix, uterus, vagina, ovaries and nearby lymph nodes. If your bladder, rectum or colon is removed, your doctor will create an artificial opening in your body (stoma) for your waste to be removed in a bag (ostomy).

• Lymph node removal. Vulvar cancer often spreads to the lymph nodes in the groin, so your doctor may remove these lymph nodes. Your doctor may also tie 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, you'll need a suction drain 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 you develop this complication, your doctor may give you compression devices or support stockings to help ease the symptoms. You'll also need to avoid scratches, sunburn and other injury to your legs because you'll have an increased risk of infection.

• Sentinel lymph node biopsy. A procedure called sentinel node biopsy may help you 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 isn't usually performed. The surgeon removes and biopsies the swollen node.

  Sentinel node biopsy is still considered experimental and isn't yet widely available.

Other complications from vulvar cancer surgery may include the development of cysts near the wounds (lymphoceles), blood clots, urinary infections, loss of sexual desire or pleasure, and painful irritation.

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's used with the hope of shrinking a large tumor so that it can be removed with less extensive surgery. Treated skin may look and feel sunburned for six to 12 months. Also, if radiation is used on the pelvic area, you may experience problems with urination and premature menopause.

Chemotherapy
Chemotherapy uses drugs, often a combination of drugs, to destroy cancer cells. It can be given through a vein, by mouth or through your skin (topically). Like radiation, chemotherapy may be used to shrink a large tumor before surgery. It's generally not used on its own because surgery is more effective, and vulvar cancers that have spread tend to be resistant to chemotherapy.

The side effects of chemotherapy may include hair loss, nausea, vomiting and fatigue. These occur because chemotherapy affects healthy cells — especially fast-growing cells in your digestive tract, hair and bone marrow — as well as cancerous ones. Not everyone has side effects, however, and there are now better ways to control some of them.

Reconstructive surgery
Treatment of vulvar cancer often involves removal of some skin from your vulva. The wound or area left behind can usually be closed without grafting skin from another area of your body. However, depending on how widespread the cancer is and how much tissue your doctor needs to remove, your doctor may perform reconstructive surgery — grafting skin from another part of your body to cover this area.

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
As many as one in 10 women experiences recurrence of vulvar cancer, so it's important to see your gynecologist at least twice a year after you finish treatment.

Living with cancer is challenging. But dealing with the physical effects of vulvar cancer and its treatment can be especially difficult. In particular, if your treatment involved vulvectomy, lymph node removal or pelvic exenteration, you may wonder how the changes in your body will affect your normal activities, your relationships and your sexuality.

It may be difficult to come to terms psychologically with the physical changes you've experienced. Although there are no easy answers for coping with vulvar cancer, the following suggestions may help:

• Find ways to make your life easier. If you have problems with incontinence or need to change an ostomy bag, for example, try to sit in the back of a movie theater, concert hall or meeting room. That way you're less conspicuous if you need to leave for the toilet. 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 for you, make rest a priority and take assistance when it's offered. Now isn't the time to turn down your friend's offer to make you dinner. And, although it may seem counterintuitive, exercise may help you feel less fatigued, as well as boost your spirits. Talk to your doctor about what kind of exercise might be right for you.

• Share your concerns with others. When you feel ready, 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. You may also find it helpful to talk to other people with vulvar cancer. They can tell you how they've coped with problems similar to the ones you're facing.

  One of the best ways to do that is to join a support group for people with vulvar or gynecologic cancers. Although support groups aren't for everyone, they can sometimes be a good resource for practical information about your disease. You may also find strength and encouragement in being with people who are facing challenges similar to yours.

  If you're interested in learning more about support groups, talk to a doctor, nurse, social worker or psychologist. They may be able to put you in touch with a group in your area. Or check your local phone book, library or cancer organization. The National Cancer Institute also can provide a list of support groups. So can the American Cancer Society at 800-ACS-2345, or 800-227-2345, or CancerCare at 800-813-HOPE, or 800-813-4673.

  After deciding to participate in a group, try it out a few times. If it doesn't seem useful or comfortable, don't feel like you have to continue.

• Don't be afraid of intimacy. Your natural reaction to changes in your body may be to avoid intimacy. Although it may not be easy, it's vitally 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. Remember that you can express your sexuality in many ways. 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've ever had.

  Intimacy issues may be even harder to address if you're not currently in a committed relationship. You may worry that no one will ever find you 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. Your doctor may be able to recommend a qualified counselor. Or call the National Cancer Institute's toll-free information line, called the Cancer Information Service. It provides access to trained counselors and accurate, up-to-date information on all aspects of living with cancer. You can reach the Cancer Information Service 24 hours a day at 800-4-CANCER, or 800-422-6237.

Avoiding risks
You can help prevent vulvar cancer by avoiding sexual behaviors that put you at risk of sexually transmitted diseases such as HPV and HIV, both conditions that increase your 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's had multiple partners. Condoms may lessen the risk of HPV transmission, but they cannot fully protect against HPV.

In addition, a vaccine against some forms of HPV is effective in preventing vulvar cancer as well as cervical and vaginal cancers. The vaccine is currently recommended for young women as they become sexually active, and its use is being studied in young men.

Not smoking also may reduce your risk of vulvar cancer.

Having regular exams
You can 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's highly treatable. The overall five-year survival rate is 96 percent when the lymph nodes aren't involved. That rate drops to 64 percent if the cancer has spread to the lymph nodes.