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. 2018 Jan;28(1):176-182.
doi: 10.1097/IGC.0000000000001131.

Invasive Paget Disease of the Vulva

Chiara Borghi, Giorgio Bogani, Antonino Ditto, Fabio Martinelli, Mauro Signorelli, Valentina Chiappa, Cono Scaffa, Stefania Perotto, Umberto Leone Roberti Maggiore, Dario Recalcati, Domenica Lorusso, Francesco Raspagliesi
Free article

Invasive Paget Disease of the Vulva

Chiara Borghi et al. Int J Gynecol Cancer. 2018 Jan.
Free article

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.

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