TY - JOUR
T1 - Vulvar Paget disease: 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, Mariëtte I. E.
AU - Slangen, Brigitte M. F.
AU - Massuger, Leon F. A. G.
AU - de Hullu, Joanne A.
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.
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85067994405&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/30458205
U2 - https://doi.org/10.1016/j.jaad.2018.11.016
DO - https://doi.org/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 -