Vulvar Cancer

Introduction

• Description: Squamous cell cancer of the vulva generally manifests as an exophytic ulcer or hyperkeratotic plaque. It may arise as a solitary lesion or develop hidden within hypertrophic or other vulvar skin changes, making diagnosis difficult and often delayed. Other malignancies (25%) such as sarcoma, melanoma, clitoral malignancy, and metastatic malignancies can also affect the vulva.

• Prevalence: Roughly 6330 new cases of and 1560 deaths from vulvar cancer each year; accounts for less than 5% of gynecologic malignancies; less common than cancer of the uterine corpus, ovary, cervix, and vagina. Lifetime risk of vulvar cancer is 0.3%. The incidence of vulvar intraepithelial neoplasia increased by approximately 50% in the last 20 years and is likely related to increased exposure to human papillomavirus (HPV).

• Predominant Age: In situ, 40–49 years; invasive, 55–70 years; median age at death is 78 years.

• Genetics: No genetic pattern.

Etiology and Pathogenesis

• Causes: Unknown, although strongly associated with HPV.

• Risk Factors: Infection with HPV (molecular analysis has detected HPV DNA [types 16, 18, and 33 predominately] in 60% of vulvar cancers), smoking, immunosuppression, lichen sclerosus, chronic irritation, northern European ancestry.

Signs and Symptoms

• Itching, irritation, cracking, or bleeding of the vulva, most common on the posterior two-thirds of the labia majus (the labia minora, perineum, clitoris, and mons are less frequently involved)

• Ulcerated exophytic lesion or hyperkeratotic plaque (late in disease)

Diagnostic Approach