Abstract
On this case report we present current theoretical knowledge about EMPD, difficulty of diagnostics, treatment and dispensarization the patients with this rare intraepithelial non-squamous neoplasia. We report a rare case of a 62 years old female patient with extramammary Paget's disease of vulva and associated well differentiated endometrial adenocarcinoma.
EMPD is a rare intraephitelial non-squamous neoplasia, which represents less then 1% vulvar tumors. Predominantly it affects white women between 60 and 80 years of age.
EMPD occurs in cutaneous areas bearing apocrine glands - vulva, perineum, perianal area, axilla, penis, scrotum and rarely region of tights or buttocks. It is characterized microscopically by the presence of specific tumor cells called Paget's cells - atypical large cells with pale clear cytoplasm and large round nuclei.