Michelle van der Linden1, Maaike H M Oonk2, Helena C van Doorn3, Johan Bulten4, Eleonora B L van Dorst5, Guus Fons6, Christianne A R Lok7, Mariëtte I E van Poelgeest8, Brigitte M F Slangen9, Leon F A G Massuger10, Joanne A de Hullu10. 1. Department of Obstetrics and Gynaecology, Radboud University Medical Center, Nijmegen, The Netherlands. Electronic address: Michelle.vanderLinden@radboudumc.nl. 2. Department of Obstetrics and Gynaecology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands. 3. Department of Gynaecology, Erasmus Medical Center Cancer Clinic, Rotterdam, The Netherlands. 4. Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands. 5. Department of Gynaecologic Oncology, University Medical Centre Utrecht, Utrecht, The Netherlands. 6. Department of Gynaecologic Oncology, Academic Medical Centre, Amsterdam, The Netherlands. 7. Department of Gynaecology, Center Gynecologic Oncology Amsterdam, Amsterdam, The Netherlands. 8. Department of Gynaecology, Leiden University Medical Centre, Leiden, The Netherlands. 9. Department of Gynaecology and Obstetrics, Maastricht University Medical Centre, Maastricht, The Netherlands. 10. Department of Obstetrics and Gynaecology, Radboud University Medical Center, Nijmegen, The Netherlands.
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 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.
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.
Authors: S Kajtezovic; Allison R Walker; B Hjalmarsson; S G Bell; E Everett; C Wong Journal: J Cancer Res Clin Oncol Date: 2022-04-17 Impact factor: 4.553
Authors: Maryam Pourmaleki; Jonathan H Young; Nicholas D Socci; Sarah Chiang; Marcia Edelweiss; Yanyun Li; Mianlei Zhang; Lev Roshal; Dennis S Chi; Klaus J Busam; Ingo K Mellinghoff; Travis J Hollmann Journal: Oncotarget Date: 2019-10-22