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

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)176-182
Number of pages7
JournalInternational journal of gynecological cancer : official journal of the International Gynecological Cancer Society
Volume28
Issue number1
DOIs
Publication statusPublished - Jan 2018

Fingerprint

Extramammary Paget's Disease
Vulva
Knowledge Management
Recurrence
Multicenter Studies
Radiotherapy
Drug Therapy
Mortality

Keywords

  • Aged
  • Chemoradiotherapy, Adjuvant
  • Chemotherapy, Adjuvant
  • Female
  • Humans
  • Middle Aged
  • Paget Disease, Extramammary/pathology
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Treatment Outcome
  • Vulvar Neoplasms/pathology

Cite this

Invasive Paget Disease of the Vulva. / Borghi, Chiara; Bogani, Giorgio; Ditto, Antonino; Martinelli, Fabio; Signorelli, Mauro; Chiappa, Valentina; Scaffa, Cono; Perotto, Stefania; Leone Roberti Maggiore, Umberto; Recalcati, Dario; Lorusso, Domenica; Raspagliesi, Francesco.

In: International journal of gynecological cancer : official journal of the International Gynecological Cancer Society, Vol. 28, No. 1, 01.2018, p. 176-182.

Research output: Contribution to journalArticle

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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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T1 - Invasive Paget Disease of the Vulva

AU - Borghi, Chiara

AU - Bogani, Giorgio

AU - Ditto, Antonino

AU - Martinelli, Fabio

AU - Signorelli, Mauro

AU - Chiappa, Valentina

AU - Scaffa, Cono

AU - Perotto, Stefania

AU - Leone Roberti Maggiore, Umberto

AU - Recalcati, Dario

AU - Lorusso, Domenica

AU - Raspagliesi, Francesco

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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.

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KW - Vulvar Neoplasms/pathology

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