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.

中文翻译：

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外阴Paget病：一项全国回顾性队列研究。

背景技术外阴Paget病（VPD）是一种罕见的皮肤病，被认为是恶性的。目的评估VPD患者的临床过程，治疗方案以及侵袭和治疗对复发和生存的影响。方法从荷兰所有高等大学医学中心的医学档案和病理报告中检索有关VPD妇女的数据。使用Kaplan-Meier曲线估算无病生存期和5年特定疾病生存期。结果分析了1991年至2016年间诊断为VPD的113例患者的数据。77％的患者患有无创性VPD。大多数妇女（65％）接受了外科手术。据报道复发率为40％。在无创性VPD的女性中，有8％发生了侵袭。无创性VPD的女性没有因疾病而死亡的报道。无创和微创VPD的5年疾病特异性生存率高于98％，但有创VPD的5年特异性生存率显着降低（50％[P <.0005]）。局限性这项研究的主要局限性是其回顾性特征和原始病理样本无法进行重新评估的事实。结论VPD极为罕见，复发率很高。大多数患者具有无创性VPD，这不会影响生存，应将其视为具有潜在侵袭潜力的慢性疾病。在浸润性疾病的情况下，存活率显着降低。局限性这项研究的主要局限性是其回顾性特征和原始病理样本无法进行重新评估的事实。结论VPD极为罕见，复发率很高。大多数患者具有无创性VPD，这不会影响生存，应将其视为具有潜在侵袭潜力的慢性疾病。在浸润性疾病的情况下，存活率显着降低。局限性这项研究的主要局限性是其回顾性特征和原始病理样本无法进行重新评估的事实。结论VPD极为罕见，复发率很高。大多数患者具有无创性VPD，这不会影响生存，应将其视为具有潜在侵袭潜力的慢性疾病。在浸润性疾病的情况下，存活率显着降低。