Paget disease of the vulva

Crit Rev Oncol Hematol. 2016 May:101:60-74. doi: 10.1016/j.critrevonc.2016.03.008. Epub 2016 Mar 4.

Abstract

In this review, we provide an overview of the clinical aspects, histopathology, molecular genetics, and treatment options for Vulvar Paget's Disease (VPD), a rare skin disease, most commonly found in postmenopausal Caucasian women. The underlying cause of VPD remains not well understood. VPD is rarely associated with an underlying urogenital, gastrointestinal or vulvar carcinoma. In approximately 25% of the cases, VPD is invasive; in these cases, the prognosis is worse than in non-invasive cases. Recurrence rates in invasive VPD are high: 33% in cases with clear margins, and even higher when surgical margins are not clear, regardless of invasion. Historically, surgical excision has been the treatment of choice. Recent studies show that imiquimod cream may be an effective and safe alternative.

Keywords: Anogenital; Extramammary Paget disease; Imiquimod; Paget disease; Perineal; Surgery; Vulvar Paget disease; Vulvar carcinoma; Vulvar neoplasm.

Publication types

  • Review

MeSH terms

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