Malignant vulvar and vaginal pathology

Abstract

Vulvar and vaginal malignant pathology are dominated by squamous cell carcinoma and its variants. In the vulva, the majority of squamous cell carcinomas are non-HPV driven (human papilloma virus), while in the vagina, HPV infection is a key driver. In both lower genital tract sites, HPV is the main cause for low and high grade squamous intraepithelial precursor lesions. Their multifocality and synchronus and metachronous existence with lesions elsewhere in the genital tract are well- established defining characteristics. Additionally, melanomas are disproportionately prevalent in the vulva when comparing the total vulvar skin surface area to that of the entire body. Melanomas can also rarely occur in the vagina. Lastly, there are four entities distinctively found in the vulvovaginal region of the female gynecologic tract: Extramammary Paget disease, aggressive angiomyxomas, embryonal rhabdomyosarcomas, and DES-related clear cell adenocarcinomas. An extensive list of different malignant entities that may occur in the vulvovaginal region are not reviewed here, but rather merely the most common.