Cancer of the vulva most often affects the inner edges of the labia majora or the labia minora – the outer and inner lips of the opening of the vagina. In the United States, vulvar cancer is not common; it accounts for about four percent of cancers of the female reproductive organs.
Most cancers of the vulva are squamous cell carcinomas, which begin in squamous cells, the main type of skin cells. Less common types of vulvar cancer include adenocarcinoma, which begins in gland cells; Paget Disease of the vulva, a condition in which adenocarcinoma cells are found in the top layer of the vulvar skin and may or may not have penetrated into the tissues below; melanoma, sarcoma, and basal cell carcinoma.
The risk of vulvar cancer increases with age. Women under 50 account for fewer than 20 percent of vulvar cancer cases, and more than half of cases occur in women over age 70. About half of all vulvar cancers are linked to infection with the high-risk types of human papilloma virus (HPV); this is particularly true in younger women. This is leading some researchers to believe there might be at least two forms of vulvar cancer. Specifically, one that is linked to HPV with features that are distinct from the other form, which is not HPV-related.
Smoking is another factor that increases the risk of developing vulvar cancer (and exacerbates the risk among women who have had HPV), as is infection with human immunodeficiency virus (HIV). Having had certain other types of cancer or pre-cancer also increases the risk for vulvar cancer. Women with the pre-cancerous condition known as vulvar intraepithelial neoplasia, or VIN, show a heightened risk, although most cases of VIN never develop into cancer. Other cancers linked to increased risk for vulvar cancer include lichen sclerosus, cervical cancer, and melanoma.
Since the exact cause of most vulvar cancer is not known, the disease is difficult to prevent and there is no standard screening test. Having regular pelvic exams may help detect vulvar and other gynecological cancers.
Common sense precautions like avoiding smoking can also help lower risk. Women can reduce their exposure to HPV by delaying sexual activity and limiting the number of sex partners, using condoms, and getting vaccinated against HPV before becoming sexually active.
Most women have no symptoms until vulvar cancer turns invasive. They then may experience unexplained and unrelieved itching; skin that appears either thicker and lighter than the normal skin around it or red, pink, and darker than the surrounding skin (those symptoms can also indicate VIN, not invasive cancer).
As the cancer grows, the tumor may be seen and felt. It sometimes takes the form of red, pink, or white bumps or could simply appear white and feel rough. Pain, burning, painful urination, abnormal bleeding and/or discharge, and open sores could also be indications of vulvar cancer and need to be seen by a physician.
Vulvar cancer is treated with a combination of surgery, radiation and chemotherapy. Recognizing the importance of female sexuality and quality of life, doctors have radically reduced the scope of surgery in recent years to minimize deformity and maintain sexual function (although extensive surgery may still be necessary in advanced cases). Topical chemotherapy may be used to treat VIN, but is not useful in treating invasive vulvar cancer. Chemotherapy has been rather ineffective at treating vulvar cancers that have spread to other organs.