Extramammary Paget's disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Extamammary Paget's disease (EMPD) is a rare cutaneous, intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis. It has predilection for apocrine gland-bearing areas: mostly the perineum, vulva, axilla, scrotum and penis. The origin of the neoplastic cells could be apocrine glands or epithelial stem cells. Extramammary paget's disease may originate in vulvar apocrine-gland bearing skin cells or as a manifestation of adjacent primary rectal, anal, or bladder adenocarcinoma. The most common site of involvement is the vulva, although perianal, perineal, scrotal and penile skin may also be affected. Extramammary Paget disease (empd) is also described as an apocrine gland tumour occurring in both a benign and a malignant form with metastatic potential. It can present as carcinoma in situ or as invasive disease that can subsequently metastasize to lymph nodes and distant sites.