Vulvar Paget disease: A national retrospective cohort study.
Adult
Aged
Aged, 80 and over
Antineoplastic Agents
/ therapeutic use
Disease-Free Survival
Female
Humans
Imiquimod
/ therapeutic use
Kaplan-Meier Estimate
Lymphatic Metastasis
Middle Aged
Neoplasm Invasiveness
Neoplasm Recurrence, Local
/ pathology
Netherlands
Paget Disease, Extramammary
/ secondary
Retrospective Studies
Survival Rate
Vulvar Neoplasms
/ pathology
Vulvectomy
Paget disease
extramammary
recurrence
survival
vulva
vulvar neoplasms
Journal
Journal of the American Academy of Dermatology
ISSN: 1097-6787
Titre abrégé: J Am Acad Dermatol
Pays: United States
ID NLM: 7907132
Informations de publication
Date de publication:
Oct 2019
Oct 2019
Historique:
received:
22
07
2018
revised:
23
10
2018
accepted:
09
11
2018
pubmed:
21
11
2018
medline:
19
2
2020
entrez:
21
11
2018
Statut:
ppublish
Résumé
Vulvar Paget disease (VPD) is a rare skin disorder that is considered premalignant. To assess the clinical course, treatment schedules, and effect of invasion and treatment on recurrence and survival in patients with VPD. Data on women with VPD were retrieved from the medical files and pathology reports in all Dutch tertiary university medical centers. Disease-free survival and 5-year disease-specific survival were estimated by using Kaplan-Meier curves. Data on 113 patients whose VPD was diagnosed between 1991 and 2016 were analyzed; 77% had noninvasive VPD. Most of the women (65%) underwent a surgical procedure. Recurrences were reported in 40%. Of the women with noninvasive VPD, 8% developed invasion. There were no disease-specific deaths reported in the women with noninvasive VPD. The 5-year disease-specific survival rate was greater than 98% in noninvasive and microinvasive VPD, but significantly worse in invasive VPD (50% [P < .0005]). The main limitations of this study are its retrospective character and the fact that original pathology samples were not available for reassessment. VPD is extremely rare, and the recurrence rates are high. Most patients have noninvasive VPD, which does not affect survival and should be considered a chronic disorder with limited invasive potential. In cases of invasive disease, survival decreases significantly.
Sections du résumé
BACKGROUND
BACKGROUND
Vulvar Paget disease (VPD) is a rare skin disorder that is considered premalignant.
OBJECTIVE
OBJECTIVE
To assess the clinical course, treatment schedules, and effect of invasion and treatment on recurrence and survival in patients with VPD.
METHODS
METHODS
Data on women with VPD were retrieved from the medical files and pathology reports in all Dutch tertiary university medical centers. Disease-free survival and 5-year disease-specific survival were estimated by using Kaplan-Meier curves.
RESULTS
RESULTS
Data on 113 patients whose VPD was diagnosed between 1991 and 2016 were analyzed; 77% had noninvasive VPD. Most of the women (65%) underwent a surgical procedure. Recurrences were reported in 40%. Of the women with noninvasive VPD, 8% developed invasion. There were no disease-specific deaths reported in the women with noninvasive VPD. The 5-year disease-specific survival rate was greater than 98% in noninvasive and microinvasive VPD, but significantly worse in invasive VPD (50% [P < .0005]).
LIMITATIONS
CONCLUSIONS
The main limitations of this study are its retrospective character and the fact that original pathology samples were not available for reassessment.
CONCLUSIONS
CONCLUSIONS
VPD is extremely rare, and the recurrence rates are high. Most patients have noninvasive VPD, which does not affect survival and should be considered a chronic disorder with limited invasive potential. In cases of invasive disease, survival decreases significantly.
Identifiants
pubmed: 30458205
pii: S0190-9622(18)32906-2
doi: 10.1016/j.jaad.2018.11.016
pii:
doi:
Substances chimiques
Antineoplastic Agents
0
Imiquimod
P1QW714R7M
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
956-962Informations de copyright
Copyright © 2018 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.