Cancer of the vulva, the outer part of the female genitalia, is rare. Vulvar cancer most often occurs in women between the ages of 55 and 85, although it can also occur in women who are younger than 40.

More than 90 percent of vulvar cancers are squamous cell carcinomas — a type of skin cancer — that develop slowly over years and are preceded by precancerous changes in the surface of your skin. A small percentage of vulvar cancers begin as melanoma. Rarely, vulvar cancers may develop in the mucus-producing glands located on the sides of the vaginal opening. The exact cause isn't known. Human papillomavirus (HPV), a sexually transmitted infection, may play a role.

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 to reduce your risk of contracting HPV infection, and you may be able to control other risk factors as well.

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

• Itching that doesn't improve
• Burning, pain and tenderness
• Bleeding that isn't from menstruation
• Skin changes, such as color changes or thickening
• A lump or open sore (ulcer)

The exact cause of vulvar cancer isn't known. Most vulvar cancers occur in women between the ages of 55 and 85 who have a mutation or defect in the p53 tumor suppressor gene — a gene that plays a role in keeping cells from becoming cancerous. These women also often have lichen sclerosus — a condition that causes the vulvar skin to become thin and itchy.

The remaining 30 percent to 50 percent of vulvar cancers occur in younger women who have the sexually transmitted infection HPV, which appears to play a key role. It may take years or decades for vulvar cancer to develop after infection with HPV. Many times these women have a precancerous skin condition called vulvar intraepithelial neoplasia in multiple areas of the vulva, and they typically are smokers.

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

• Age. The majority of women with vulvar cancers, 85 percent, are older than 50 years of age. And half of women with vulvar cancers are older than 70 when first diagnosed with the cancer. However, 15 percent of new diagnoses are 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, which affects the body's immune system, 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 don't develop 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.
• Melanoma. If you have a family or personal history of this serious type of skin cancer anywhere on your body, you're at increased risk of a vulvar melanoma.
• Immunosuppression. Medications that suppress your immune system may also increase your risk of vulvar cancer if you have an established HPV infection. Some women may not even realize they are infected with HPV until they use these medications. Immunosuppression with drugs such as steroids is commonly used in people with organ transplants.

Because an early diagnosis of vulvar cancer increases the likelihood of successful treatment, it's important that you see your doctor if you experience any of the signs and symptoms of vulvar cancer.

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

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 suggest a noncancerous 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.

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, he or she may choose one of 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 4 millimeters across.

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:

• Cytoscopy. Using a lighted tube, your doctor examines the inside surface of your bladder. More advanced 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.
• Proctoscopy. In this test, your doctor uses a lighted tube to inspect your rectum and removes tissue samples from any abnormal-looking areas for microscopic analysis.
• Pelvic examination under anesthesia. With 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, CT scan and CAT scan — is an X-ray technique that produces more detailed images of your internal organs than do conventional X-ray exams. Conventional X-ray exams produce two-dimensional images. But CT uses an X-ray-sensing unit that rotates around your body, and a large computer to create cross-sectional images (like slices) of the inside of your body. A CT scan can help your doctor see if cancer has spread to your liver or other organs. Some CT scans require you to ingest a contrast medium before the scan. A contrast medium blocks X-rays and appears white on images, which can help emphasize structures in your body.
• Magnetic resonance imaging (MRI). This test uses a magnetic field and radio waves to create cross-sectional images. Most MRI machines are large, cylindrical-shaped magnets. The strong magnetic field is produced by passing an electric current through wire loops or coils, which are located inside a protective housing. Other coils in the housing send and receive radio waves. When you're in the machine, your body produces very faint signals in response to the radio waves. These signals are detected by coils within the machine, or by additional coils designed to surround a specific body part needing examination. A computer then processes the signals and generates an image. The collected signals create a composite, three-dimensional representation of your body.
• 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 tissues do 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.

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.

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 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 of the normal tissue all the way around it.

• 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.

• 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 weeks.

  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.

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 therapy uses high-energy X-rays to kill cancer cells. The radiation is given outside the body and is usually used only to treat the lymph nodes in the groin and pelvis, not the vulva itself. Sometimes it's used to shrink a large tumor so that it can later be removed with less extensive surgery. The skin in the treated areas may look and feel sunburned for six to 12 months. Also, if radiation is used on the pelvis area, you may experience problems with premature menopause and urination.

Chemotherapy
Chemotherapy uses drugs to destroy cancer cells. You may take these drugs through a vein, by mouth or through your skin (topically). 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.

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. Not smoking may also reduce your risk of vulvar cancer.

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 curable. According to the American Cancer Society, the overall five-year survival rate is 90 percent when the lymph nodes aren't involved. The overall five-year survival rate drops to 50 percent to 70 percent if cancer has spread to the lymph nodes.

In addition, a new vaccine against HPV, which was approved by the Food and Drug Administration in 2006, is effective in preventing vulvar cancer as well as cervical cancer.

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 exteneration, 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.

• 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 the same challenges you are.

  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, you don't 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.