Invasive Paget Disease of the Vulva

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OBJECTIVE: Extramammary Paget disease of the vulva (EPDV) is a rare occurrence with an indolent and relapsing course. Progression to invasion occurs in 4% to 19% of cases. The aim of this study is to report clinical-pathological features and outcomes of patients treated for invasive EPDV.

METHODS: Data of consecutive patients treated between 2000 and 2017 for invasive EPDV were reviewed.

RESULTS: Among 79 patients with EPDV, 10 (12.7%) presented a microinvasive or invasive form at first diagnosis or during follow-up. All of them underwent upfront radical surgery; 7 (70%) received subsequent radiotherapy, chemotherapy, or both. The mortality rate was 40%. The recurrence rate after treatment for invasive forms was 60%, with a mean time to first recurrence of 20 (range, 5-36) months.

CONCLUSIONS: Our study confirms that invasive EPDV remains a rare gynecological neoplasm with a poor prognosis. Multicentre trials or well-organized prospective data collection could improve the knowledge about the management of invasive EPDV.

Original languageEnglish
Pages (from-to)176-182
Number of pages7
JournalInternational journal of gynecological cancer : official journal of the International Gynecological Cancer Society
Issue number1
Publication statusPublished - Jan 2018


  • Aged
  • Chemoradiotherapy, Adjuvant
  • Chemotherapy, Adjuvant
  • Female
  • Humans
  • Middle Aged
  • Paget Disease, Extramammary/pathology
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Treatment Outcome
  • Vulvar Neoplasms/pathology


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