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Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light!
Background: Vulvar Paget’s disease (VPD) is defined as a neoplasm of epithelial origin, mostly in postmenopausal women. Due to the extreme rarity of VPD, limited data about recommended treatment options are available. Surgical excision has been the treatment of choice although in the recent decade m...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9861591/ https://www.ncbi.nlm.nih.gov/pubmed/36675761 http://dx.doi.org/10.3390/jpm13010100 |
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author | Della Corte, Luigi Cafasso, Valeria Conte, Carmine Cuomo, Lara Giampaolino, Pierluigi Lavitola, Giada Bifulco, Giuseppe |
author_facet | Della Corte, Luigi Cafasso, Valeria Conte, Carmine Cuomo, Lara Giampaolino, Pierluigi Lavitola, Giada Bifulco, Giuseppe |
author_sort | Della Corte, Luigi |
collection | PubMed |
description | Background: Vulvar Paget’s disease (VPD) is defined as a neoplasm of epithelial origin, mostly in postmenopausal women. Due to the extreme rarity of VPD, limited data about recommended treatment options are available. Surgical excision has been the treatment of choice although in the recent decade medical treatments have been proposed. Methods: A systematic computerized search of the literature was performed in the main electronic databases (MEDLINE, EMBASE, Web of Science, PubMed, and Cochrane Library), from 2003 to September 2022, in order to analyze all medical and surgical strategies used for the treatment of VPD. Results: Thirty-four articles were included in this review with findings as follows: 390 patients were treated with medical or other conservative treatment while 2802 patients were treated surgically; 235/434 (54%) patients had a complete response, 67/434 (15%) a partial response, 10/434 (2.3%) a stable disease, 3/434 (0.7%) disease progress, 3/434 (0.7%) died of the disease, 55/434 (13%) died of other causes during follow up while 7/434 (1.6%) had to stop topical treatments with 5% imiquimod cream because of side effects; 239/434 patients (55%) had a recurrence and 11/434 (2.5%) were lost to follow-up. The length of follow-up was variable, according to the different studies analyzed. Conclusion: VPD is a chronic disease with a high recurrence rate and low mortality. There are no significant differences in recurrence rates in patients who undergo surgery and those who do not and the margin status at the time of primary surgery and recurrence. Several surgical and medical approaches providing both local control of the disease and minimal tissue damage have been developed. Clock mapping, a recent preoperative vulvo-vaginal workup tool, can predict the invasiveness and the extension of VPD. However, to date, due to the different treatment options available and in the absence of a global consensus, it is critical to tailor treatments to individual patient characteristics and biopsy histopathologic findings, to ensure the best type of therapy. |
format | Online Article Text |
id | pubmed-9861591 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-98615912023-01-22 Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! Della Corte, Luigi Cafasso, Valeria Conte, Carmine Cuomo, Lara Giampaolino, Pierluigi Lavitola, Giada Bifulco, Giuseppe J Pers Med Review Background: Vulvar Paget’s disease (VPD) is defined as a neoplasm of epithelial origin, mostly in postmenopausal women. Due to the extreme rarity of VPD, limited data about recommended treatment options are available. Surgical excision has been the treatment of choice although in the recent decade medical treatments have been proposed. Methods: A systematic computerized search of the literature was performed in the main electronic databases (MEDLINE, EMBASE, Web of Science, PubMed, and Cochrane Library), from 2003 to September 2022, in order to analyze all medical and surgical strategies used for the treatment of VPD. Results: Thirty-four articles were included in this review with findings as follows: 390 patients were treated with medical or other conservative treatment while 2802 patients were treated surgically; 235/434 (54%) patients had a complete response, 67/434 (15%) a partial response, 10/434 (2.3%) a stable disease, 3/434 (0.7%) disease progress, 3/434 (0.7%) died of the disease, 55/434 (13%) died of other causes during follow up while 7/434 (1.6%) had to stop topical treatments with 5% imiquimod cream because of side effects; 239/434 patients (55%) had a recurrence and 11/434 (2.5%) were lost to follow-up. The length of follow-up was variable, according to the different studies analyzed. Conclusion: VPD is a chronic disease with a high recurrence rate and low mortality. There are no significant differences in recurrence rates in patients who undergo surgery and those who do not and the margin status at the time of primary surgery and recurrence. Several surgical and medical approaches providing both local control of the disease and minimal tissue damage have been developed. Clock mapping, a recent preoperative vulvo-vaginal workup tool, can predict the invasiveness and the extension of VPD. However, to date, due to the different treatment options available and in the absence of a global consensus, it is critical to tailor treatments to individual patient characteristics and biopsy histopathologic findings, to ensure the best type of therapy. MDPI 2023-01-01 /pmc/articles/PMC9861591/ /pubmed/36675761 http://dx.doi.org/10.3390/jpm13010100 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Della Corte, Luigi Cafasso, Valeria Conte, Carmine Cuomo, Lara Giampaolino, Pierluigi Lavitola, Giada Bifulco, Giuseppe Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! |
title | Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! |
title_full | Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! |
title_fullStr | Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! |
title_full_unstemmed | Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! |
title_short | Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light! |
title_sort | medical and surgical strategies in vulvar paget disease: let’s throw some light! |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9861591/ https://www.ncbi.nlm.nih.gov/pubmed/36675761 http://dx.doi.org/10.3390/jpm13010100 |
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