Vulvar cancer accounts for about 4% of cancers in the female reproductive organs and 0.6% of all cancers in women. The American Cancer Society estimates that in 2005, about 3,870 cancers of the vulva will be diagnosed in the United States and about 870 women will die of this cancer. Cancer of the vulva accounts for 4.7% of malignant neoplasms in the genital tract. It is the fourth most frequent gynecologic cancer.

Most vulvar carcinomas occur in older women, with more than 50% of the patients being 60 to 79 years of age. Invasive vulvar carcinomas are being seen with increasing frequency in younger patients, however, with 15% of vulvar cancers arising in women under the age of 40 years (

Figure 100-1). This increased frequency in younger patients may be attributed to an increase in the number of sexual partners or venereal viral infections within the population.

Several epidemiologic studies suggest a sexually transmitted origin for carcinoma of the vulva. Condyloma acuminatum associated with human papillomavirus (HPV) has been noted in many patients with premalignant and malignant vulvar disease.

It has been estimated that in the United States, over 1 million women each year develop perineal warts and that as many as 10% are infected with HPV. Currently, HPV types 6 and 11 are most frequently found in benign vulvar warts, and HPV types 16, 18, 31, 33, and 45 are more frequently associated with intraepithelial neoplasia or invasive carcinoma. HPV can be found in approximately 50% of vulvar carcinomas; the tumors are often multifocal and associated with vulvar dysplasias. HPV-negative tumors are often found in older women.

Although epidemiologic evidence strongly suggests a viral cause, other associations have been implied as well. Factors such as granulomatous diseases of the vulva, diabetes, hypertension, and obesity also have been associated with vulvar carcinoma, but perhaps this is because of the usually advanced age of patients. A case-control study by Mabuchi and colleagues found that domestic servants, or those working in laundry or cleaning plants, have an increased risk of vulvar carcinoma, thus suggesting an environmental component.

The association of carcinoma in situ with invasive carcinoma of the vulva indicates a continuum from preinvasive to invasive carcinoma. The progression of vulvar intraepithelial neoplasia to invasive carcinoma has been estimated to be on the order of 6%. Progression, however, may differ between younger and older patients. Some authors suggest that the multifocal carcinoma in situ of women in their thirties or forties may not be as likely to progress as that seen in older women.

Rowley KC, Gallion HH, Donalson ES