Vulvar Paget disease: A national retrospective cohort study

Linden, M. van der; Oonk, M.H.M.; Doorn, H.C. van; Bulten, J.; Dorst, E.B.L. van; Fons, G.; Lok, C.A.R.; Poelgeest, M.I.E. van; Slangen, B.M.F.; Massuger, L.F.A.G.; Hullu, J.A. de

doi:doi:10.1016/j.jaad.2018.11.016

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])...Show moreBackground: 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.Show less

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Vulvar Paget disease: A national retrospective cohort study