Invasive Paget Disease of the Vulva.

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Invasive Paget Disease of the Vulva.

Int J Gynecol Cancer. 2018 Jan;28(1):176-182

Authors: Borghi C, Bogani G, Ditto A, Martinelli F, Signorelli M, Chiappa V, Scaffa C, Perotto S, Leone Roberti Maggiore U, Recalcati D, Lorusso D, Raspagliesi F

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.

PMID: 28976448 [PubMed - indexed for MEDLINE]

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