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Invasive Paget Disease of the Vulva
  1. Chiara Borghi, MD*,,
  2. Giorgio Bogani, MD, PhD,
  3. Antonino Ditto, MD,
  4. Fabio Martinelli, MD,
  5. Mauro Signorelli, MD,
  6. Valentina Chiappa, MD,
  7. Cono Scaffa, MD, PhD,
  8. Stefania Perotto, MD,
  9. Umberto Leone Roberti Maggiore, MD,
  10. Dario Recalcati, MD,
  11. Domenica Lorusso, MD, PhD and
  12. Francesco Raspagliesi, MD
  1. * Institute of Obstetrics and Gynecology, Department of Morphology, Surgery and Experimental Medicine, University of Ferrara, Ferrara; and
  2. Department of Gynecologic Oncology, IRCCS National Cancer Institute, Milan, Italy.
  1. Address correspondence and reprint requests to Dr. Chiara Borghi, MD, Gynecologic Oncology Unit, National Cancer Institute, Via Venezian 1, 20133 Milan, Italy. E-mail: borghi.chr{at}gmail.com.

Abstract

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.

  • Extramammary Paget disease of the vulva
  • Invasive vulvar Paget disease
  • Vulvar neoplasm
  • Surgery

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Footnotes

  • The authors declare no conflicts of interest.

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