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A 63-year-old black woman presents with a “knot” on her labia majora (Figures 87-1 and 87-2). She is a smoker but is otherwise healthy. The lesion is occasionally pruritic but is generally asymptomatic. She found it approximately 6 months ago, and it has been slowly increasing in size. There is no significant family history of cancer. On physical exam she is found to have exophytic condyloma acuminata around the introitus and a growth labelled vulvar intraepithelial neoplasia (VIN) that the patient called a “knot.” A 3-mm punch biopsy is performed and demonstrates VIN III. The patient is referred to gynecologic oncology.

Figure 87-1

Patient with multiple exophytic condyloma and vulvar intraepithelial neoplasia III. The large clitoral hood is an incidental finding unrelated to the vulvar intraepithelial neoplasia. (Courtesy of Hope Haefner, MD.)

Figure 87-2

Close-up of the same patient with vulvar intraepithelial neoplasia III on the labia majora. (Courtesy of Hope Haefner, MD.)

Vulvar dysplasia and cancer is less common than cervical cancer. It is associated with high-risk human papillomavirus (HPV) infection, but not to the same extent as cervical disease.

  • Vulvar cancer is the fourth most common gynecologic cancer (following cancer of the endometrium, ovary, and cervix) and accounts for 5% of lower female genital tract malignancies.1 There are approximately 3900 new cases and 870 deaths each year in the United States from this disease.1
  • Worldwide, vulvar cancer is rare, especially in developing countries. Approximately 27,000 cases are reported annually, making the incidence rate between 1 and 1.5 per 100,000 women.2
  • Seventy-five percent of VIN cases occur in premenopausal women, with no racial predisposition.
  • Although the rate of invasive vulvar carcinoma has remained stable in the past two decades, the incidence of in situ disease (VIN) has more than doubled. This may be the result of improved surveillance and treatment of VIN, or the apparent increase in cases of VIN in younger women.3

  • VIN is the associated preneoplastic condition that is associated with the loss of epithelial cell maturation and nuclear abnormalities.
  • The cervix, vagina, vulva, anus, and lower 3 cm of rectal mucosa is derived from the embryonic cloaca. Most squamous intraepithelial lesions in this area affect multiple anatomic sites.
  • HPV 16 is estimated to contribute to approximately 77% of VIN lesions.4
  • The risk of neoplastic progression appears to be lower with VIN than with cervical intraepithelial neoplasia. VIN I probably has minimal malignant potential, but VIN III often progresses to invasive cancer if left untreated (see “Biopsy” below).5

  • Risk factors are similar to those for vaginal and cervical dysplasia:6
    • High-risk HPV infection.
    • Cigarette smoking.
    • Altered immune status.

Clinical Features

  • The vulva is best ...

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