TY - JOUR T1 - Invasive Paget Disease of the Vulva JF - International Journal of Gynecologic Cancer JO - Int J Gynecol Cancer SP - 176 LP - 182 DO - 10.1097/IGC.0000000000001131 VL - 28 IS - 1 AU - Chiara Borghi AU - Giorgio Bogani AU - Antonino Ditto AU - Fabio Martinelli AU - Mauro Signorelli AU - Valentina Chiappa AU - Cono Scaffa AU - Stefania Perotto AU - Umberto Leone Roberti Maggiore AU - Dario Recalcati AU - Domenica Lorusso AU - Francesco Raspagliesi Y1 - 2018/01/01 UR - http://ijgc.bmj.com/content/28/1/176.abstract N2 - 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. ER -