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PATIENT STORY

A 63-year-old black woman presents with a "knot" on her labia majora (Figures 89-1 and 89-2). 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 papule that the patient called a "knot." A 3-mm punch biopsy is performed and demonstrates vulvar intraepithelial neoplasia (VIN) III (high-grade squamous intraepithelial lesion [HSIL]). The patient is referred to gynecologic oncology.

FIGURE 89-1

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

FIGURE 89-2

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

INTRODUCTION

Vulvar intraepithelial neoplasia is a premalignant condition of the vulva. Vulvar dysplasia and cancer are less common than cervical cancer. It is associated with high-risk human papillomavirus (HPV) infection, but not to the same extent as cervical disease.

In the 2004 International Society for the Study of Vulvar Diseases nomenclature, VIN was broken into two main categories1:

  • VIN usual type—Dysplasia highly associated with high-risk HPV infection. There is no evidence that what was previously called VIN I is a cancer precursor, so the term VIN was restricted to high-grade lesions (formerly termed VIN II and VIN III) in which treatment to prevent progression to cancer is indicated.2 The term condyloma acuminatum is now used for lesions previously referred to as VIN I.

  • VIN differentiated type—Lesions that are not high-risk HPV associated and have a worse prognosis.

In 2012, the Lower Anogenital Squamous Terminology (LAST) project of the American Society for Colposcopy and Cervical Pathology and the College of American Pathology described changes in the terminology in which condyloma acuminatum is referred to as vulvar low-grade squamous intraepithelial lesion (LSIL) and VIN II, III is referred to as vulvar HSIL.3

EPIDEMIOLOGY

  • 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.4 There are approximately 3900 new cases and 870 deaths each year in the United States from this disease.4

  • 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.5

  • Seventy-five percent of ...

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