Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, University of Washington School of Medicine, Seattle, WA 98195, USA.
Gynecol Oncol. 2011 Feb;120(2):296-9. doi: 10.1016/j.ygyno.2010.10.019.
To characterize clinical features of vulvar Paget's disease, and examine the quantity of immunosuppressive regulatory T-cells in vulvar Paget's tissue.
Vulvar Paget's cases from 1992 to 2007 from two institutions were identified by pathology database search. Regulatory T-cells were identified with FOXP3 immunohistochemistry and quantified at the dermal-epidermal junction using image analysis software. Thirteen non-neoplastic inflammatory cases were stained for comparison.
Cases included 33 women treated for primary vulvar Paget's, and 7 referred at recurrence. Of the 24 primary cases with greater than 5 months follow-up, recurrence was documented in 12/24(50%). Eight women (20%) recurred multiple times, but no recurrences were invasive. Significantly more patients with positive margins developed recurrent disease (82% vs 23%, p=0.01). Secondary neoplasms occurred in 10/40(25%). FOXP3+ cells at the dermal-epidermal junction were quantified in 29 primary and 13 recurrent tissue samples. FOXP3+ cells were absent in surrounding normal vulvar skin. FOXP3+ cells averaged 66/HPF in primary vulvar Paget's and 66/HPF in recurrent Paget's, compared to 22/HPF in non-neoplastic inflammatory cases (p=0.0003, p=0.001). Primary cases with positive surgical margins had more FOXP3+ cells than those with negative margins (85 vs 49, p=0.01). Recurrent cases with positive margins had more FOXP3+ cells than negative cases (84 vs 33, p=0.06). FOXP3 levels in primary specimens were higher in cases which recurred (78 vs 35, p=0.02).
Increased regulatory T-cells may be associated with more extensive cases of vulvar Paget's disease that result in positive surgical margins and are associated with recurrence of disease, suggesting immunosuppression as a key factor.
描述外阴派杰氏病的临床特征，并研究外阴派杰氏组织中免疫抑制调节性 T 细胞的数量。
通过病理数据库检索，确定了 1992 年至 2007 年来自两个机构的外阴派杰氏病病例。采用 FOXP3 免疫组化法鉴定调节性 T 细胞，并使用图像分析软件在外皮-真皮交界处对其进行定量。对 13 例非肿瘤性炎症病例进行染色作为对照。
共纳入 33 例接受原发性外阴派杰氏病治疗的女性患者，7 例为复发患者。在 24 例原发性病例中有大于 5 个月随访记录的患者中，12/24（50%）发生复发。8 例（20%）患者多次复发，但均无浸润性复发。切缘阳性的患者更易发生疾病复发（82% vs 23%，p=0.01）。40 例患者中有 10 例（25%）发生继发性肿瘤。在 29 例原发性和 13 例复发性组织样本中定量了真皮-表皮交界处的 FOXP3+细胞。周围正常外阴皮肤中无 FOXP3+细胞。原发性外阴派杰氏病的 FOXP3+细胞平均为 66/高倍视野（HPF），复发性外阴派杰氏病为 66/HPF，而非肿瘤性炎症病例为 22/HPF（p=0.0003，p=0.001）。切缘阳性的原发性病例的 FOXP3+细胞数多于切缘阴性的病例（85 对 49，p=0.01）。切缘阳性的复发性病例的 FOXP3+细胞数多于切缘阴性的病例（84 对 33，p=0.06）。复发的原发性病例的 FOXP3 水平高于未复发的病例（78 对 35，p=0.02）。
调节性 T 细胞增多可能与外阴派杰氏病更广泛的病例有关，这些病例导致切缘阳性并与疾病复发有关，提示免疫抑制是一个关键因素。
