Extramammary Paget´s disease of the vulva - a case report
Kolářová, D.; Havránková, A.; Líbalová, P.; Frühaufová, S.; Vernerová, Z.; Kučera, E.
Ceska Gynekologie 79(5): 394-398
2014
ISSN/ISBN: 1210-7832 PMID: 25472459 Document Number: 671834
Analysis of one case of vulvar extramammary Paget´s disease (EMPD) and associated well differentiated endometrial adenocarcinoma.Desing: A case report. 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. extramammary Paget´s disease, EMPD, Paget´s cells, vulvectomy, imiquimod 5%, photodynamic therapy, imunotherapy, radiotherapy.