Vulvar cancer is a rare disease that occurs mainly in elderly women. The vulva refers to the external female genitalia, which includes the labia, the opening of the vagina, the clitoris, and the space between the vagina and anus (perineum). There are two pairs of labia (a Latin term meaning lips). The labia meet to protect the openings of the vagina and the tube that connects to the bladder (urethra). The outer, most prominent folds of skin are called labia majora, and the smaller, inner skin folds are called labia minora. Vulvar cancer can affect any part of the female genitalia, but usually affects the labia.

Approximately 70% of vulvar cancers involve the labia (usually the labia majora), 15% to 20% involve the clitoris, and 15% to 20% involve the perineum. For approximately 5% of the cases, the cancer is present at more than one location. For approximately 10% of the cases, so much of the vulva is affected by cancer that the original location cannot be determined. Vulvar cancer can spread to nearby structures including the anus, vagina, and urethra.

Most vulvar cancers are squamous cell carcinomas. Squamous cells are the main cell type of the skin. Squamous cell carcinoma often begins at the edges of the labia majora or labia minora or the area around the vagina. This type of cancer is usually slow-growing and may begin with a precancerous condition referred to as vulvar intraepithelial neoplasia (VIN), or dysplasia. This means that precancerous cells are present in the surface layer of skin.

Other, less common types of vulvar cancer are melanoma, basal cell carcinoma, adenocarcinomas, Paget's disease of the vulva, and tumors of the connective tissue under the skin. Melanoma, a cancer that develops from the cells that produce the pigment that determines the skin's color, can occur anywhere on the skin, including the vulva. Melanoma is the second most common type of vulvar cancer, and accounts for 5% to 10% of the cases. Half of all vulvar melanomas involve the labia majora. Basal cell carcinoma, which is the most common type of cancer that occurs on parts of the skin exposed to the sun, very rarely occurs on the vulva. Adenocarcinomas develop from glands, including the glands at the opening of the vagina (Bartholin's glands) that produce a mucus-like lubricating fluid.

Vulvar cancer is most common in women over 50 years of age. The median age at diagnosis is 65 to 70 years old. Additional risk factors for vulvar cancer include having multiple sexual partners, cervical cancer, and the presence of chronic vaginal and vulvar inflammations. This type of cancer is often associated with sexually transmitted diseases.

Vulvar cancer is most common in women who are between the ages of 65 and 75 years. In the United States there are approximately 3,000 new cases of vulvar cancer diagnosed each year. Vulvar cancer accounts for only 1% of all cancers in women. Approximately 5% of all gynecologic cancers occur on the vulva. For unknown reasons, the incidence of vulvar cancer seems to be rising.

Cancer is caused when the normal mechanisms that control cell growth become disturbed, causing the cells to continually grow without stopping. This is usually the result of damage to the DNA in the cell. Although the cause of vulvar cancer is unknown, studies have identified several risk factors for vulvar cancer. These include:

• Vulvar intraepithelial neoplasia (VIN). This abnormal growth of the surface cells of the vulva can sometimes progress to cancer.
• Infection with human papillomavirus (HPV). This virus is sexually transmitted and can cause genital warts. Although HPV DNA can be detected in most cases of vulvar intraepithelial neoplasia, it is detected in fewer than half of all cases of vulvar cancer. Therefore, the link between HPV infection and vulvar cancer is unclear. As of 2001, it is theorized that two classes of vulvar cancer exist: one that is associated with HPV infection and one that is not.
• Herpes simplex virus 2 (HSV2). This sexually transmitted virus is also associated with increased risk for vulvar cancer.
• Cigarette smoking. Smoking in combination with infection by HPV or HSV2 was found to be a particularly strong risk factor for vulvar cancer.
• Infection with human immunodeficiency virus (HIV). This virus, which causes AIDS, decreases the body's immune ability, leaving it vulnerable to a variety of diseases, including vulvar cancer.
• Chronic vulvar inflammation. Long term irritation and inflammation of the vulva and vagina, which may be caused by poor hygiene, can increase the risk of vulvar cancer.
• Abnormal Pap smears. Women who have had abnormal Pap smears are at an increased risk of developing vulvar cancer.
• Chronic immunosuppression. Women who have had long-term suppression of their immune system caused by disease (such as certain cancers) or medication (such as those taken after organ transplantation) have an increased risk of developing vulvar cancer.

The hallmark symptom of vulvar cancer is itching (pruritus), which is experienced by 90% of the women afflicted by this cancer. The cancerous lesion is readily visible. Unfortunately, because of embarrassment or denial, it is not uncommon for women to delay medical assessment of vulvar abnormalities. Any abnormalities should be reported to a gynecologist.

If squamous cell vulvar cancer is present, it may appear as a raised red, pink, or white bump (nodule). It is often accompanied by pain, bleeding, vaginal discharge, and painful urination. Malignant melanoma of the vulva usually appears as a pigmented, ulcerated growth. Other types of vulvar cancer may appear as a distinct mass of tissue, sore and scaly areas, or cauliflower-like growths that look like warts.

A gynecological examination will be used to observe the suspected area. During this examination, the physician may use a special magnifying instrument called a colposcope to view the area better. Additionally, the area may be treated with a dilute solution of acetic acid, which causes some abnormal areas to turn white, making them easier to see. During this examination, if any area is suspected of being abnormal, a tissue sample (biopsy) will be taken. The biopsy can be performed in the doctor's office with the use of local anesthetic. A wedge-shaped piece of tissue, which contains the suspect lesion with some surrounding normal skin and the underlying skin layers and connective tissue, will be removed. Small lesions will be removed in their entirety (excisional biopsy). The diagnosis of cancer depends on a microscopic analysis of this tissue by a pathologist.

The diagnosis for vulvar cancer will determine how advanced the cancer is and how much it has spread. This is determined by the size of the tumor and how deep it has invaded the surrounding tissue and organs, such as the lymph nodes. It will also be determined if the cancer has metastasized, or spread to other organs. Tests used to determine the extent of the cancer include x ray and computed tomography scan (CT scan). Endoscopic examination of the bladder (cystoscopy) and/or rectum (proctoscopy) may be performed if it is suspected that the cancer has spread to these organs.

The International Federation of Gynecology and Obstetrics (FIGO) has adopted a surgical staging system for vulvar cancer. The stage of cancer is determined after surgery. The previous clinical staging system for vulvar cancer is no longer used. Vulvar cancer is categorized into five stages (0, I, II, III, and IV) which may be further subdivided (A and B) based on the depth or spread of cancerous tissue. The FIGO stages for vulvar cancer are:

• Stage 0. Vulvar intraepithelial neoplasia.
• Stage I. Cancer is confined to the vulva and perineum. The lesion is less than 2 cm (about 0.8 in) in size.
• Stage II. Cancer is confined to the vulva and perineum. The lesion is larger than 2 cm (larger than 0.8 in) in size.
• Stage III. Cancer has spread to the vagina, urethra, anus, and/or the lymph nodes in the groin (inguinofemoral).
• Stage IV. Cancer has spread to the bladder, bowel, pelvic bone, pelvic lymph nodes, and/or other parts of the body.

Treatment for vulvar cancer will depend on its stage and the patient's general state of health. Surgery is the mainstay of treatment for most cases of vulvar cancer.

The primary treatment for stage I and stage II vulvar cancer is surgery to remove the cancerous lesion and possibly the inguinofemoral lymph nodes. Removal of the lesion may be done by laser, to burn off a minimal amount of tissue, or by scalpel (local excision), to remove more of the tissue. The choice will depend on the severity of the cancer. If a large area of the vulva is removed, it is called a vulvectomy. Radical vulvectomy removes the entire vulva. A vulvectomy may require skin grafts from other areas of the body to cover the wound and make an artificial vulva. Because of the significant morbidity and the psychosexual consequences of radical vulvectomy, there is a trend toward minimizing the extent of cancer excision. The specific inguinofemoral lymph node that would receive lymph fluid from the cancerous lesion, known as the sentinel node, may be exposed for examination (lymph node dissection) or removed (lymphadenectomy), especially in cases in which the cancerous lesion has invaded to a depth of more than 1 mm. Surgery may also be followed by chemotherapy and/or radiation therapy to kill additional cancer cells.

Surgical treatment of stage III and stage IV vulvar cancer is much more complex. Extensive surgery would be necessary to completely remove the cancerous tissue. Surgery would involve excision of pelvic organs (pelvic exenteration), radical vulvectomy, and lymphadenectomy. Because this extensive surgery comes with a substantial risk of complications, it may be possible to treat advanced vulvar cancer with minimal surgery by using radiation therapy and/or chemotherapy as additional treatment (adjuvant therapy).

An intraoperative technique that is used to identify the sentinel node in breast cancer and melanoma is being applied to vulvar cancer. This technique, called lymphoscintigraphy, is performed during surgical treatment of vulvar cancer and allows the surgeon to immediately identify the sentinel node. A radioactive compound (technetium 99m sulfur colloid) is injected into the cancerous lesion approximately two hours prior to surgery. This injection causes little discomfort, so local anesthesia is not required. During surgery, a radioactivity detector is used to locate the sentinel node and any other nodes to which cancer has spread. Though still in the experimental stage, vulvar lymphoscintigraphy shows promise in reducing morbidity and hospital length of stay.

The most common complication of vulvectomy is the development of a tumor-like collection of clear liquid (wound seroma). Other surgical complications include urinary tract infection, wound infection, temporary nerve injury, fluid accumulation (edema) in the legs, urinary incontinence, falling or sinking of the genitals (genital prolapse), and blood clots (thrombus).

Radiation therapy uses high-energy radiation from x rays and gamma rays to kill the cancer cells. The skin in the treated area may become red and dry and may take as long as a year to return to normal. Fatigue, upset stomach, diarrhea, and nausea are also common complaints of women having radiation therapy. Radiation therapy in the pelvic area may cause the vagina to become narrow as scar tissue forms. This phenomenon, known as vaginal stenosis, makes intercourse painful.

Chemotherapy uses anticancer drugs to kill the cancer cells. The drugs are given by mouth (orally) or intravenously. They enter the bloodstream and can travel to all parts of the body to kill cancer cells. Generally, a combination of drugs is given because it is more effective than a single drug in treating cancer. The side effects of chemotherapy are significant and include stomach upset, vomiting, appetite loss, hair loss, mouth or vaginal sores, fatigue, menstrual cycle changes, and premature menopause. There is also an increased chance of infections.

Although alternative and complementary therapies are used by many cancer patients, very few controlled studies on the effectiveness of such therapies exist. Mind-body techniques such as prayer, biofeedback, visualization, meditation, and yoga have not shown any effect in reducing cancer but can reduce stress and lessen some of the side effects of cancer treatments. Clinical studies of hydrazine sulfate found that it had no effect on cancer and even worsened the health and well- being of the study subjects. One clinical study of the drug amygdalin (Laetrile) found that it had no effect on cancer. Laetrile can be toxic and has caused death. Shark cartilage, although highly touted as an effective cancer treatment, is an improbable therapy that has not been the subject of clinical study.

The American Cancer Society has found that the “metabolic diets” pose serious risk to the patient. The effectiveness of the macrobiotic, Gerson, and Kelley diets and the Manner metabolic therapy has not been scientifically proven. The FDA was unable to substantiate the anticancer claims made about the popular Cancell treatment.

There is no evidence for the effectiveness of most over-the-counter herbal cancer remedies. However, some herbals have shown an anticancer effect. As shown in clinical studies, Polysaccharide krestin, from the mushroom Coriolus versicolor, has significant effectiveness against cancer. In a small study, the green alga Chlorella pyrenoidosa has been shown to have anticancer activity. In a few small studies, evening primrose oil has shown some benefit in the treatment of cancer.

Factors that are correlated with disease outcome include the diameter and depth of the cancerous lesion, involvement of local lymph nodes, cell type, HPV status, and age of the patient. Vulvar cancers that are HPV positive have a better prognosis than those that are HPV negative. The 5-year survival rate is 98% for stage I vulvar cancer and 87% for stage II vulvar cancer. The survival rate drops steadily as the number of affected lymph nodes increases. The survival rate is 75% for patients with one or two, 36% for those with three or four, and 24% for those with five or six involved lymph nodes. The previous statistics were obtained from studies of patients who received surgical treatment only and cannot be used to determine survival rates when adjuvant therapy is employed.

Vulvar cancer can spread locally to encompass the anus, vagina, and urethra. Because of the anatomy of the vulva, it is not uncommon for the cancer to spread to the local lymph nodes. Advanced stages of vulvar cancer can affect the pelvic bone. The lungs are the most common site for vulvar cancer metastasis. Metastasis through the blood (hematogenous spread) is uncommon.

The risk of vulvar cancer can be decreased by avoiding risk factors, most of which involve lifestyle choices. Specifically, to reduce the risk of vulvar cancer, women should not smoke and should refrain from engaging in unsafe sexual behavior. Good hygiene of the genital area to prevent infection and inflammation may also reduce the risk of vulvar cancer.

Because vulvar cancer is highly curable in its early stages, women should consult a physician as soon as a vulvar abnormality is detected. Regular gynecological examinations are necessary to detect precancerous conditions that can be treated before the cancer becomes invasive. Because some vulvar cancer is a type of skin cancer, the American Cancer Society also recommends self-examination of the vulva using a mirror. If moles are present in the genital area, women should employ the ABCD rule:

• Asymmetry. A cancerous mole may have two halves of unequal size.
• Border irregularity. A cancerous mole may have ragged or notched edges.
• Color. A cancerous mole may have variations in color.
• Diameter. A cancerous mole may have a diameter wider than 6 mm (1/4 in).