Carcinoma of the vulva: epidemiology and pathogenesis

Vulvar squamous carcinoma is an uncommon neoplasm that afflicts a spectrum of women and has been associated with granulomatous vulvar diseases, human papillomaviruses (HPVs), and chronic inflammatory disorders of the vulva. This review summarizes the epidemiologic, histopathologic, and viral data supporting the division of invasive vulvar carcinomas into distinct subsets. Although HPVs have received attention as etiologic agents, histopathologic and viral data indicate that a substantial proportion of vulvar carcinomas in this country may not be related to a veneareally transmitted agent. One of the principal challenges is to produce studies integrating the various disciplines in order to place HPV in proper perspective and develop strategies to identify women at risk for vulvar carcinomas that are not associated with this virus.