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 | |
| Publication status | Published - Jan 2018 |
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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 journal › Article
}
TY - JOUR
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
PY - 2018/1
Y1 - 2018/1
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.
KW - Aged
KW - Chemoradiotherapy, Adjuvant
KW - Chemotherapy, Adjuvant
KW - Female
KW - Humans
KW - Middle Aged
KW - Paget Disease, Extramammary/pathology
KW - Radiotherapy, Adjuvant
KW - Retrospective Studies
KW - Treatment Outcome
KW - Vulvar Neoplasms/pathology
U2 - 10.1097/IGC.0000000000001131
DO - 10.1097/IGC.0000000000001131
M3 - Article
C2 - 28976448
VL - 28
SP - 176
EP - 182
JO - International Journal of Gynecological Cancer
JF - International Journal of Gynecological Cancer
SN - 1048-891X
IS - 1
ER -