Vulvar Paget disease: a national retrospective cohort study

Publication date: Available online 17 November 2018

Source: Journal of the American Academy of Dermatology

Author(s): Michelle van der Linden, Maaike H.M. Oonk, Helena C. van Doorn, Johan Bulten, Eleonora B.L. van Dorst, Guus Fons, Christianne A.R. Lok, Mariëtte I.E. van Poelgeest, Brigitte M.F. Slangen, Leon F.A.G. Massuger, Joanne A. de Hullu

Abstract

Background

Vulvar Paget disease (VPD) is a rare skin disorder, considered premalignant.

Objective

To assess the clinical course, treatment schedules and the 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 centres. Disease free survival (DFS) and 5-year disease specific survival (DSS) were estimated using Kaplan-Meier curves.

Results

Data of 113 patients diagnosed between 1991 and 2016 were analysed. Seventy-seven percent had non-invasive VPD. Most women underwent surgery (65%). Recurrences were reported in 40%. Of women with non-invasive VPD 8% developed invasion. There were no disease specific deaths reported in women with non-invasive VPD. The 5 year DSS was over 98% in non-invasive and micro-invasive VPD, but significantly worse in invasive VPD: 50% (p<0.0005).

Limitations

The main limitations of this study are its retrospective character and that original pathology samples were not available for reassessment.

Conclusions

VPD is extremely rare and recurrence rates are high. Most patients have non-invasive VPD, which does not affect survival and should be considered a chronic disorder with a limited invasive potential. In case of invasive disease survival decreases significantly.

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