According to the American Cancer Society, up to half of basaloid and warty squamous cell cancers of the vulva appear to be associated with human papillomavirus (HPV) infection. Women who develop these cancers tend to be younger and are often smokers.

The second process by which vulvar cancers develop does not involve HPV infection. Squamous cell cancers of the vulva that aren’t linked to HPV infection usually affect women over age 55 and may develop from a condition called differentiated vulvar intraepithelial neoplasia, and may be associated with mutations of the p53 tumor suppressor gene. This gene plays an important role in preventing cells from becoming cancerous, and mutations may weaken that defense. It is not known how these gene mutations occur.

There are other known risk factors for vulvar cancer. Having these risk factors does not mean a woman will develop vulvar cancer, and some women who do have the disease have no risk factors.

Vulvar cancer risk factors include:

Human papillomavirus (HPV) infection

HPV infection has been linked to more than half of all vulvar cancers. For more on HPV, refer to the subsection below.

Age

Vulvar cancer risk increases with age. Fewer than 20 percent of cases are diagnosed in women under 50, while more than half of all cases occur in women older than 70. The average age for women diagnosed with invasive vulvar cancer is 70. For non-invasive vulvar cancer, the average age is 50.

Tobacco use

Smoking is a major risk factor for vulvar cancer. For women who have a history of high-risk HPV infection, smoking significantly raises the risk of vulvar cancer.

HIV infection

Infection with human immunodeficiency virus (HIV) damages the immune system, making HPV infection more likely and raising the risk for vulvar cancer.

Lichen sclerosis

This condition causes the skin in the vulva to become thin and itchy, and appears to slightly increase vulvar cancer risk. About 4 percent of women who have lichen sclerosis develop vulvar cancer.

Other genital cancers

Women who have cervical cancer tend to have a higher risk of vulvar cancer. This is likely due to the shared risk factors for the diseases, including HPV infection and smoking.

Melanoma or atypical moles

Women who have had melanoma skin cancer or atypical moles elsewhere on their skin have a higher risk for vulvar melanoma. A family history of melanoma also raises the risk.

Vulvar intraepithelial neoplasia (VIN)

VIN is the medical term for the precancerous changes that occur in the top layer of vulvar skin before squamous cell cancer develops. VIN can last for several years before the cells become cancer. In most cases, women with VIN will not develop cancer, but there is no way to know if it will or not, so medical care is important. The two types include usual-type VIN and differentiated-type VIN.

• Usual-type VIN occurs in younger women and is caused by HPV infection. It can become the basaloid or warty subtypes of squamous cell carcinoma.
• Differentiated-type VIN tends to occur in older women with no HPV link. It can become the keratinizing subtype of squamous cell cancer.

Having any of these risk factors does not mean you will develop vulvar cancer. But if you’re concerned about your risk, talk with your doctor. If you have family members who have had vulvar cancer, consider talking with one of our genetic counselors.

More on HPV

HPV is a group of more than 150 related viruses which can infect cells lining the genitals, anus, mouth and throat. It’s spread from one person to another during skin-to-skin contact, including sex. Infection with a high-risk type of HPV, such as HPV 16 or 18, may cause precancerous cell changes. If not treated, these cells may become cancerous.

Up to 80 percent of men and women who have had sex have HPV. In 9 out of 10 cases, the body’s immune system clears the virus on its own, and most women with HPV will not develop vulvar cancer. But if the infection becomes chronic, the risk of vulvar cancer increases. A vaccine series is available for HPV and is recommended for pre-teens between the ages of 11 and 12. In some cases, the vaccine may be recommended up to age 26.

There are no screening tests for vulvar cancer, but having annual pelvic exams can make it easier to detect precancerous changes or early-stage vulvar cancer, when treatment is most likely to be successful. Between exams, practice awareness — know the signs and symptoms of vulvar cancer and call your doctor if you notice anything unusual.

Preventive factors

Vulvar cancer cannot be completely prevented, but you can take the following steps to help with vulvar cancer prevention:

• Avoid tobacco
• Have annual pelvic exams
• Have HPV tests as recommended by your physician
• Ask your doctor about an HPV vaccine
• Use condoms during sex to protect against sexually transmitted diseases
• Have precancerous conditions treated

Your Scripps MD Anderson cancer care team will customize your treatment plan based on several factors, including:

• The cancer stage
• Whether cancer has spread
• Your age and overall health

Treatment for precancerous vulvar intraepithelial neoplasia (VIN) or early-stage vulvar cancer usually involves surgery. More advanced cases may include chemotherapy or radiation therapy.

Topical therapy

In some cases, physicians may be able to treat early precancerous vulvar cell changes with medicine applied to the area.

Vulvar cancer surgery

Depending on the stage of the cancer and how far it may have spread, Scripps physicians may use several surgical procedures to treat vulvar cancer. These include:

• Laser surgery is when the physician uses a laser beam to destroy abnormal cells.
• Excision is when the cancer and about a ½ inch of skin on each side of it are removed.
• Vulvectomy is when some or all of the vulva is removed. After this procedure, a plastic surgeon may perform reconstructive surgery.
• Lymph node dissection is when surgeons remove lymph nodes from the pelvic region to see if the cancer has spread. This can be performed through a minimally invasive laparoscopic surgery.
• Pelvic exenteration is the most extensive surgical approach and is used for advanced vulvar cancer. It involves the removal of the vulva and lymph nodes, and may include the removal of the cervix, uterus, bladder, vagina, rectum and possibly part of the colon, based on how far the cancer has spread.

With pelvic exenteration, the following organs may be involved:

• If the bladder is removed, the surgeon may use a piece of intestine to form a new bladder, and urine may be drained through a catheter (tube) into a small plastic bag worn outside of the body.
• If the rectum and part of the colon are removed, you may have an opening on the abdomen that allows solid waste (stool) to pass into a small bag worn on the outside of the body, called a colostomy. In some cases, the surgeon may be able to reconnect the colon, so no colostomy is needed.
• If the vagina is removed, the surgeon may be able to make a new one from other tissue.

Chemotherapy

Chemotherapy may be used to shrink tumors before surgery, or when the disease has spread beyond the pelvis. It may be used in combination with radiation therapy.

Radiation therapy

Radiation therapy may be used to treat vulvar cancers that have spread, or to shrink large tumors before surgery. Scripps radiation oncologists use advanced radiation therapy techniques that precisely target tumors while minimizing radiation exposure to nearby healthy tissues and organs.

Some vulvar cancer treatment plans may involve clinical trials. Talk with your physician about whether a clinical trial is right for you.

For a list of clinical trial that are currently enrolling patients, see our current list of clinical trials.

Your Scripps MD Anderson cancer team will develop a customized treatment plan outlining the treatments and therapies we recommend for your care. Before you begin treatment, you and your physician will review your plan together and discuss any questions or concerns you may have.

Your vulvar cancer treatment plan generally includes:

• Family and medical history
• A summary of your cancer diagnosis and staging information
• Diagnostic testing completed (e.g., imaging, biopsy, lab tests)
• Plans for surgery, radiation, chemotherapy and/or other treatments
• Potential side effects of treatments
• Contact information for your cancer care team members

Along with helping you understand your course of treatment and encouraging discussion between you and your physician, your plan helps your team coordinate your care. You also can share your treatment plan with other providers who may be involved in your medical care.

Your vulvar cancer care team includes health and medical professionals from a wide range of specialties. Scripps MD Anderson will customize your team to ensure you have the expertise and support you need.

Your team may include:

• Gynecologists
• Gynecologic surgeons
• Surgeons and surgical oncologists
• Radiation oncologists
• Medical oncologists
• Registered nurses
• Nurse navigators
• Dietitians
• Oncology social workers

Visit your cancer care team for more on Scripps MD Anderson's multidisciplinary approach to treatment.

Patient education is an integral part of understanding and coping with your cancer diagnosis and treatment. To stay informed, we encourage our patients, along with their caregivers and family members, to:

• Bookmark the Scripps glossary of cancer terms for easy referencing.
• Download the appointment form and list of medications form on our resources page.
• Consult your oncology team for educational materials and a list of trusted online sources beyond the Scripps site. 

Navigating cancer might seem overwhelming — especially with so much information online. To ensure you receive the most accurate details, always look to your multidisciplinary team of cancer care experts first.