PT - JOURNAL ARTICLE 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 TI - Invasive Paget Disease of the Vulva AID - 10.1097/IGC.0000000000001131 DP - 2018 Jan 01 TA - International Journal of Gynecologic Cancer PG - 176--182 VI - 28 IP - 1 4099 - http://ijgc.bmj.com/content/28/1/176.short 4100 - http://ijgc.bmj.com/content/28/1/176.full SO - Int J Gynecol Cancer2018 Jan 01; 28 AB - 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.