[Extramammary Paget´s disease of the vulva - a case report].

OBJECTIVE: Analysis of one case of vulvar extramammary Paget´s disease (EMPD) and associated well differentiated endometrial adenocarcinoma.Desing: A case report.

METHODS AND RESULTS: 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.

CONCLUSIONS: 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.

KEYWORDS: extramammary Paget´s disease, EMPD, Paget´s cells, vulvectomy, imiquimod 5%, photodynamic therapy, imunotherapy, radiotherapy.