Vulvar Paget disease: A national retrospective cohort study
van der Linden, Michelle; Oonk, Maaike H. M.; van Doorn, Helena C.; Bulten, Johan; van Dorst, Eleonora B. L.; Fons, Guus; Lok, Christianne A. R.; van Poelgeest, Mariette I. E.; Slangen, Brigitte M. F.; Massuger, Leon F. A. G.; de Hullu, Joanne A.
(2019) Journal of the American Academy of Dermatology, volume 81, issue 4, pp. 956 - 962
(Article)
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
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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.
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Keywords: extramammary, Paget disease, recurrence, survival, vulva, vulvar neoplasms, Dermatology, Journal Article
ISSN: 0190-9622
Publisher: Mosby Inc.
Note: 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.
(Peer reviewed)