Vulvar Paget disease: A national retrospective cohort study.
J Am Acad Dermatol
; 81(4): 956-962, 2019 Oct.
Article
en En
| MEDLINE
| ID: mdl-30458205
ABSTRACT
BACKGROUND:
Vulvar Paget disease (VPD) is a rare skin disorder that is considered premalignant.OBJECTIVE:
To assess the clinical course, treatment schedules, and effect of invasion and treatment on recurrence and survival in patients with VPD.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:
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:
The main limitations of this study are its retrospective character and the fact that original pathology samples were not available for reassessment.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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Neoplasias de la Vulva
/
Enfermedad de Paget Extramamaria
/
Recurrencia Local de Neoplasia
Tipo de estudio:
Etiology_studies
/
Observational_studies
Límite:
Adult
/
Aged
/
Aged80
/
Female
/
Humans
/
Middle aged
País/Región como asunto:
Europa
Idioma:
En
Revista:
J Am Acad Dermatol
Año:
2019
Tipo del documento:
Article