Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is characterized by slow grow and high recurrence rates. This article reviews the epidemiological, clinical, morphological, genetic and treatment features of EMPD of the vulva reported in recent years.

Department of Pathology Clinical Research and Practical Center for Specialized Oncological care Saint Petersburg Russian Federation; Department of Pathology Medical Faculty Saint Petersburg State University Russian Federation; Department of Pathology Saint Petersburg Medico Social Institute St Petersburg Russian Federation

Sikl's Department of Pathology Medical Faculty in Pilsen Charles University Prague Pilsen Czech Republic; Bioptical Laboratory Pilsen Czech Republic

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