Paget`s Disease of the Vulva: Report of Four Cases

Lim, G S; Park, N H; Lee, C M; Kim, Y B; Kim, J W; Song, Y S; Park, I A; Kang, S B; Lee, H P

Abstract

Extramammary Paget`s disease of the vulva is an uncommon vulvar neoplasm that is a slowly progressive intraepithelial carcinoma and develops in an apocrine gland-bearing areas of the body. Several hundred cases have been described worldwide, however the precise incidence remains unclear. Paget`s disease of the vulva most often presents as an intraepithelial lesion, but underlying adenocarcinoma with this process in up to 20% of cases. A second synchronous or metachronous primary neoplasia is associated with extramammary Paget`s disease in about 30% of patients. The most common presenting symptom is vulvar pruritus, and typical finding is erythematous, eczematous lesions of the vulva. Vulvar Paget`s disease is often multifocal, and histologic evidence of disease is frequently greater than is clinically apparent. So, frozen-section surgical margin evaluation is utilized as a guide to extent of excision. Treatment of Paget`s disease requires wide local excision and if there is an underlying adenocarcinoma, radical vulvectomy is required, which should be combined with at least an ipsilateral inguinal-femoral lymphadenectomy. Due to its rarity and different disease patterns, we present a clinicopathologic study of four cases seen at our hospital from 1988 to 1998.