Invasive Paget's disease of the vulva and perianal region: a case report - Revista Brasileira de Ginecologia e Obstetrícia

Extramammary Paget’s disease (EPD) is an uncommon neoplasic condition observed mostly in areas with numerous apocrine and or eccrine glands. In the woman it is most commonly seen on the vulva, although it can occur in other locations. Vulvar Paget’s disease (VPD) can be classified into primary, of cutaneous origin, and secondary, of extracutaneous origin, with significant clinical e prognostic implications. Clinically VPD begins insidiously with pruritus and burning sensation. The lesion appears as a solitary patch with an eczematous, erythematous and squamous surface. This is a report of a case of a 72-year-old patient with an erythematous slightly thickened patch lesion with spots of erosion involving both the right and the left majus and minus labia, the clitoris, the pubic region, and the perineal and perianal regions. The operation performed included radical vulvectomy and bilateral inguinal lymphadenectomy. The histopathology revealed invasive Paget’s disease. Immunohistochemical methods showed positive Paget cells for CEA, EMA, and cytokeratin pan. Pathogenesis and diagnosis of EPD is discussed, with differential diagnosis and reference to immunohistochemical methods. Recurrence rate is 30%, even with margin control. Experience with EPD is limited and long-term follow-up is required to exclude recurrence of the disease and development of an associated cancer.