Gynecology and Obstetrics 1, Department of Surgical Sciences, City of Health and Science, University of Torino, Torino, Italy.
Gynecology and Obstetrics 1, Department of Surgical Sciences, City of Health and Science, University of Torino, Torino, Italy.
Surg Oncol. 2021 Sep;38:101581. doi: 10.1016/j.suronc.2021.101581. Epub 2021 Apr 16.
To evaluate clinico-pathological features, treatments and survival outcomes of vulvar Paget's disease (VPD).
We retrospectively reviewed VPD diagnosed between 1983 and 2018 at the Department of Surgical Sciences, Sant'Anna Hospital, Turin. Clinico-pathological characteristics and surgical treatment outcomes were investigated according to the depth of invasion.
A total of 122 patients were identified. Eighty-seven patients were diagnosed with intraepithelial VPD, 22 with microinvasive (<=1 mm) VPD and 16 with invasive VPD. The median follow-up was 94.6 months (interquartile range 25th-75th, 26-120). Most of patients 95/122 (77%) were treated by surgery. Local recurrence was observed in 69/95 (73%) patients without significant difference between the 3 groups (p = 0.33), however, total vulvectomy showed better local control in microinvasive and invasive VPD than in intraepithelial tumors. At 120 months the cancer-specific survival was 100% for intraepithelial and microinvasive VPD versus 31% for invasive VPD (log-rank p = <0.0001) Age ≥65 years (OR: 4.17 CI 1.12-15.5, p = 0.03) and VPD's area ≥15 cm (OR: 5.83 CI 1.75-19.3, p = 0.004) were associated with risk of invasiveness.
Microinvasive VPD has an identical prognosis to intraepithelial VPD, suggesting the omission of lymphadenectomy or adjuvant treatments are safe in this subset of patients. We recommend caution to propose medical treatment in patients who are ≥65 years old and with wide tumor area, as they are at the greatest risk of invasiveness.
评估外阴派杰氏病（VPD）的临床病理特征、治疗方法和生存结果。
我们回顾性分析了 1983 年至 2018 年在都灵圣安娜医院外科科学系诊断为 VPD 的患者。根据浸润深度研究了临床病理特征和手术治疗结果。
共确定了 122 例患者。87 例患者诊断为上皮内 VPD，22 例患者诊断为微浸润（<=1mm）VPD，16 例患者诊断为浸润性 VPD。中位随访时间为 94.6 个月（四分位间距 25-75，26-120）。大多数患者（95/122，77%）接受了手术治疗。122 例患者中有 69 例（73%）出现局部复发，3 组间无显著差异（p=0.33），然而，微浸润和浸润性 VPD 中全外阴切除术比上皮内肿瘤有更好的局部控制。120 个月时，上皮内和微浸润性 VPD 的癌症特异性生存率为 100%，而浸润性 VPD 为 31%（对数秩检验 p<0.0001）。年龄≥65 岁（OR：4.17，CI 1.12-15.5，p=0.03）和 VPD 面积≥15cm（OR：5.83，CI 1.75-19.3，p=0.004）与侵袭性相关。
微浸润性 VPD 的预后与上皮内 VPD 相同，提示在这组患者中，省略淋巴结切除术或辅助治疗是安全的。我们建议在年龄≥65 岁和肿瘤面积较大的患者中谨慎采用药物治疗，因为这些患者侵袭性风险最大。
