Extramammary Paget's disease

Extramammary Paget's disease (EMPD) is an uncommon but distinctive tumor. The lesion is defined as an intra-epidermal neoplasm. The lesion may be accompanied by an invasive adenocarcinoma or in situ adenocarcinoma of apocrine glands. Visceral carcinoma may also coexist or develop. Definitive diagnosis requires biopsy of the lesion and immunohistochemical staining. In most cases of noninvasive or minimally invasive EMPD, surgical resection with clear margins and careful follow-up are recommended, since the recurrence rate is high. We review the literature and report two cases of EMPD, one involving the perineal-scrotal area and the other involving the perianal area.