TY - JOUR
T1 - Vulvar Paget disease
T2 - A national retrospective cohort study
AU - van der Linden, Michelle
AU - Oonk, Maaike H. M.
AU - van Doorn, Helena C.
AU - Bulten, Johan
AU - van Dorst, Eleonora B. L.
AU - Fons, Guus
AU - Lok, Christianne A. R.
AU - van Poelgeest, Mariette I. E.
AU - Slangen, Brigitte M. F.
AU - Massuger, Leon F. A. G.
AU - de Hullu, Joanne A.
N1 - Funding Information:
The authors would like to thank the collaborating pathology departments for identifying all the women in their local pathology database: M.C.G. Bleeker, MD, PhD (Academic Medical Centre, Amsterdam); K. van de Vijver, MD, PhD, (Antonie van Leeuwenhoek Hospital); P.C. Ewing-Graham, MD (Erasmus Medical Center); T. Bosse, MD, PhD (Leiden University Medical Centre); L.F.S. Kooreman, MD (Maastricht University Medical Centre); A. Siebers, PhD (Radboud University Medical Center, Nijmegen); H. Hollema, MD, PhD (University Medical Centre Groningen); and G.N. Jonges, MD, PhD (Maastricht University Medical Centre). Funding sources: None.
Publisher Copyright:
© 2018 American Academy of Dermatology, Inc.
PY - 2019/10
Y1 - 2019/10
N2 - 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.
AB - 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.
KW - extramammary
KW - Paget disease
KW - recurrence
KW - survival
KW - vulva
KW - vulvar neoplasms
KW - OUTCOMES
KW - RECURRENCE
KW - SURGERY
KW - MARGINS
U2 - 10.1016/j.jaad.2018.11.016
DO - 10.1016/j.jaad.2018.11.016
M3 - Article
C2 - 30458205
SN - 0190-9622
VL - 81
SP - 956
EP - 962
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 4
ER -