RT Journal Article
SR Electronic
T1 Invasive Paget Disease of the Vulva
JF International Journal of Gynecologic Cancer
JO Int J Gynecol Cancer
FD BMJ Publishing Group Ltd
SP 176
OP 182
DO 10.1097/IGC.0000000000001131
VO 28
IS 1
A1 Chiara Borghi
A1 Giorgio Bogani
A1 Antonino Ditto
A1 Fabio Martinelli
A1 Mauro Signorelli
A1 Valentina Chiappa
A1 Cono Scaffa
A1 Stefania Perotto
A1 Umberto Leone Roberti Maggiore
A1 Dario Recalcati
A1 Domenica Lorusso
A1 Francesco Raspagliesi
YR 2018
UL http://ijgc.bmj.com/content/28/1/176.abstract
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.