Extramammary Paget disease of the vulva

Semin Diagn Pathol. 2021 Jan;38(1):50687. doi: 10.1053/j.semdp.2020.09.007. Epub 2020 Sep 8.

Abstract

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.

Keywords: Extramammary Paget disease; Pathology; Treatment; Vulva.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Female
  • Humans
  • Paget Disease, Extramammary / diagnosis
  • Paget Disease, Extramammary / epidemiology
  • Paget Disease, Extramammary / pathology*
  • Prognosis
  • Vulvar Neoplasms / diagnosis
  • Vulvar Neoplasms / epidemiology
  • Vulvar Neoplasms / pathology*