Paget's disease of the vulva: A challenge for the gynaecologist

J Gynecol Obstet Hum Reprod. 2021 Jan;50(1):101896. doi: 10.1016/j.jogoh.2020.101896. Epub 2020 Aug 20.

Abstract

Paget's disease of the vulva is a rare form of extramammary Paget's disease mainly affecting postmenopausal women. Its pathophysiology remains largely unknown. Up to fairly recently, the only treatment for this disease was surgery, often mutilating the vulva, with significant psychosexual repercussions without the assurance of complete therapeutic efficacy. New therapeutic approaches -topical treatments, radiotherapy or chemotherapy- have emerged in recent years but lack consensual guidelines. We present a literature review of the recent results published in this field.

Keywords: Cancer; Extramammary Paget disease; Imiquimod; Paget's disease; Surgery; Vulva.

Publication types

  • Systematic Review

MeSH terms

  • Administration, Topical
  • Antineoplastic Agents / therapeutic use
  • Diagnosis, Differential
  • Female
  • Humans
  • Imiquimod / therapeutic use
  • Lasers, Gas / therapeutic use
  • Neoplasm Invasiveness
  • Neoplasm Recurrence, Local
  • Paget Disease, Extramammary / pathology
  • Paget Disease, Extramammary / therapy*
  • Photochemotherapy
  • Prognosis
  • Radiotherapy Dosage
  • Vulvar Neoplasms / pathology
  • Vulvar Neoplasms / therapy*

Substances

  • Antineoplastic Agents
  • Imiquimod