The vulva is the outside part of the female reproductive system that leads to the vagina. It includes inner and outer labia (Latin for lips) that protect the vaginal opening and, just above it, the opening of the urethra, the tube that carries urine from the bladder. At the front of the vagina, the labia minora meet to form a fold or small hood of skin called the prepuce. Beneath it is the clitoris.

Cancer of the vulva, or vulvar cancer, most often affects the inner edges of the labia majora or the labia minora. Less often, cancer occurs on the clitoris or in Bartholin glands (small mucus-producing glands on either side of the vaginal opening).

These are the most common types of vulvar cancer are:

• Squamous cell carcinomas: These cancers usually form slowly over many years and are usually preceded by pre-cancerous changes that may last for several years. Dysplasia is often used as a term to describe these pre-cancerous changes. More than 90% of vulvar cancers are this type.
• Melanoma: This cancer type develops from the pigment-producing cells that determine the skin’s color. About 5% to 8% of melanomas in women occur on the vulva, usually on the labia minora and clitoris.
• Adenocarcinomas: These cancers develop from glands and account for a small percentage of vulvar cancers.
• Paget disease of the vulva: In this condition, adenocarcinoma cells are found in the vulvar skin. Between 20% and 25% of patients with vulvar Paget disease also have an invasive adenocarcinoma of a Bartholin gland or sweat gland. In the remaining 75% to 80%, the malignant cells are found only in the skin’s top layer and do not involve the tissues under that layer.
• Sarcomas: These tumors of the connective tissues develop under the skin and tend to grow rapidly. Unlike other cancers of the vulva, vulvar sarcomas can occur at any age, including in childhood. They account for less than 2% of vulvar cancers.

Risk Factors

Risk factors increase the odds of getting a disease but do not always mean a disease will occur. Here are some vulvar cancer risk factors:

• Age: Almost 85% of women with vulvar cancer are over age 50, and half are over age 70 at the time of diagnosed. However, 15% of new patients are under age 40.
• Human papillomavirus (HPV) infection: HPV infection is thought to be responsible for most vulvar cancers in younger women. Different HPV types cause different warts in various parts of the body, including the female and male genital organs. Since HPV infection is passed during sexual contact, people who have had many sexual partners or with partners who have had many sexual partners are at increased risk. Infection with high-risk HPVs often produces no visible signs until pre-cancerous changes or cancer develops.
• History of cervical cancer: Having cervical cancer or cervical pre-cancerous conditions (cervical intraepithelial neoplasia or cervical dysplasia) increases risk of developing vaginal squamous cell cancer. This is because cervical and vaginal cancers have similar risk factors, such as HPV infection.
• Smoking: Women who smoke and have a history of genital warts are at increased risk of developing vulvar cancer. Women infected with a high-risk HPV also have a much higher risk of developing vulvar cancer if they smoke.
• HIV infection: Because the virus that causes AIDS damages the body’s immune system, it makes women more susceptible to persistent HPV infections, which may increase the risk of pre-cancerous vulvar changes and vulvar cancer.
• Vulvar intraepithelial neoplasia (VIN): Women with VIN have an increased risk of developing invasive vulvar cancer. Although most cases of VIN never progress to cancer, it is not possible to tell which will, so medical follow-up is important.
• History of melanoma or atypical moles on nonvulvar skin: Women with a family history of melanoma or dysplastic nevi (atypical moles) elsewhere on the body are at risk for developing melanoma on the vulva.
• Other genital cancers: Women with vulvar cancer also have a higher risk of cervical cancer. The likely reason for this association is the role of HPV infection in causing both of these cancers.

Symptoms of Vulvar Cancer

Some signs and symptoms of early invasive vulvar cancer are similar to those of symptomatic dysplasia. The most common symptoms include the following:

• A red, pink, or white bump or bumps with a wart-like or raw surface
• An area of the vulva that appears white and feels rough
• Persistent itching and a growth
• Pain, burning, painful urination, bleeding, and discharge not associated a normal menstrual period
• An ulcer that persists for more than a month
• Appearance of a darkly pigmented growth or a change in a mole that has been present for years (symptom of a melanoma)
• A distinct mass on either side of the opening to the vagina (symptom of a Bartholin gland carcinoma)
• Soreness and a red, scaly area (symptoms of Paget disease of the vulva)

Many of these symptoms may result from less serious conditions and don’t necessarily indicate cancer. Discuss persistent issues with a doctor, and remember, the sooner patients receive a correct diagnosis, the more effective treatment can be.

Early Detection and Prevention

Although there are no standard screenings for this disease, there are steps women can take to protect themselves:

• Get women’s health exams yearly.
• Discuss family history with your doctor.
• See your doctor right away if you have symptoms.

Diagnosis of Vulvar Cancer

Although certain signs and symptoms may strongly suggest vulvar cancer, many can be caused by benign (non-cancerous) conditions. If your doctor suspects a problem, he or she may request a biopsy.

In a biopsy, a local anesthetic is used to numb the area and a small piece of tissue is removed. At a laboratory, a pathologist views the tissue under a microscope to determine if cancer is present.

Staging and Treatment

Staging is the process of determining the extent of a cancer and where it is in the body. Vulvar cancers at varying stages have different prognoses and treatments.

Treatment options depend on the cancer’s stage, the patient’s general health, plans for bearing children, and other personal considerations. These are common treatments for vulvar cancer:

• Surgery
• Radiation therapy
• Chemotherapy

Sometimes combinations of these treatment methods are used. The patient and her cancer health care team will determine the best treatment options.