Extramammary Paget's Disease
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.
Sir James Paget first described Paget's disease of nipple in 1874 in 15 patients with underlying ductal breast cancers. In 1889, Crocker described the first case of extramammary Paget's disease affecting the scrotum and penis. Extramammary Paget's disease (EMPD) is a rare form of adenocarcinoma observed mostly in areas with numerous apocrine or eccrine glands. It is presumed to be a variant of the epithelial carcinoma, with potential to develop from or to be the cause of an underlying adenocarcinoma. Even though for more than 100 years various theories have been suggested regarding the exact pathogenesis of EMPD and Paget's cells, a consensus has not been reached. We report two cases of this rare disease and briefly review available literature.
Abstract
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.
Introduction
Sir James Paget first described Paget's disease of nipple in 1874 in 15 patients with underlying ductal breast cancers. In 1889, Crocker described the first case of extramammary Paget's disease affecting the scrotum and penis. Extramammary Paget's disease (EMPD) is a rare form of adenocarcinoma observed mostly in areas with numerous apocrine or eccrine glands. It is presumed to be a variant of the epithelial carcinoma, with potential to develop from or to be the cause of an underlying adenocarcinoma. Even though for more than 100 years various theories have been suggested regarding the exact pathogenesis of EMPD and Paget's cells, a consensus has not been reached. We report two cases of this rare disease and briefly review available literature.
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