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

doi:10.1097/IGC.0000000000001131

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

Fondazione IRCCS Istituto Nazionale dei Tumori

Research output: Contribution to journal › Article › peer-review

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 language	English
Pages (from-to)	176-182
Number of pages	7
Journal	International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
Volume	28
Issue number	1
DOIs	https://doi.org/10.1097/IGC.0000000000001131
Publication status	Published - Jan 2018

Keywords

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

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10.1097/IGC.0000000000001131

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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. (2018). Invasive Paget Disease of the Vulva. International journal of gynecological cancer : official journal of the International Gynecological Cancer Society, 28(1), 176-182. https://doi.org/10.1097/IGC.0000000000001131

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 journal › Article › peer-review

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 2018, 'Invasive Paget Disease of the Vulva', International journal of gynecological cancer : official journal of the International Gynecological Cancer Society, vol. 28, no. 1, pp. 176-182. https://doi.org/10.1097/IGC.0000000000001131

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. / Invasive Paget Disease of the Vulva. In: International journal of gynecological cancer : official journal of the International Gynecological Cancer Society. 2018 ; Vol. 28, No. 1. pp. 176-182.

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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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author = "Chiara Borghi and Giorgio Bogani and Antonino Ditto and Fabio Martinelli and Mauro Signorelli and Valentina Chiappa and Cono Scaffa and Stefania Perotto and {Leone Roberti Maggiore}, Umberto and Dario Recalcati and Domenica Lorusso and Francesco Raspagliesi",

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AU - Borghi, Chiara

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AU - Martinelli, Fabio

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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.

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.

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KW - Radiotherapy, Adjuvant

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KW - Treatment Outcome

KW - Vulvar Neoplasms/pathology

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JO - International Journal of Gynecological Cancer

JF - International Journal of Gynecological Cancer

SN - 1048-891X

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Invasive Paget Disease of the Vulva

Abstract

Keywords

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