Department of Obstetrics and Gynaecology, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Obstetrics and Gynaecology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands.
J Am Acad Dermatol. 2019 Oct;81(4):956-962. doi: 10.1016/j.jaad.2018.11.016. Epub 2018 Nov 17.
Vulvar Paget disease (VPD) is a rare skin disorder that is considered premalignant.
To assess the clinical course, treatment schedules, and effect of invasion and treatment on recurrence and survival in patients with VPD.
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.
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]).
The main limitations of this study are its retrospective character and the fact that original pathology samples were not available for reassessment.
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.
外阴 Pagets 病（VPD）是一种罕见的皮肤疾病，被认为具有潜在恶性。
评估 VPD 患者的临床病程、治疗方案以及侵袭和治疗对复发和生存的影响。
从所有荷兰三级大学医学中心的病历和病理报告中检索到 VPD 女性患者的数据。使用 Kaplan-Meier 曲线估计无病生存率和 5 年疾病特异性生存率。
分析了 1991 年至 2016 年间诊断为 VPD 的 113 名患者的数据；77%的患者为非侵袭性 VPD。大多数女性（65%）接受了手术。报告有 40%的患者复发。非侵袭性 VPD 的女性中有 8%发展为侵袭性。非侵袭性 VPD 的女性没有疾病特异性死亡。非侵袭性和微侵袭性 VPD 的 5 年疾病特异性生存率大于 98%，但侵袭性 VPD 的生存率显著较差（50%[P<.0005]）。
本研究的主要局限性在于其回顾性特征以及无法获得原始病理样本进行重新评估。
VPD 极为罕见，复发率较高。大多数患者为非侵袭性 VPD，不影响生存，应被视为具有有限侵袭性的慢性疾病。侵袭性疾病的情况下，生存率显著下降。
