Department of Pathology, Duke University Medical Center, Durham, NC, USA.
Int J Gynecol Pathol. 2010 Jan;29(1):69-78. doi: 10.1097/PGP.0b013e3181b1cc5e.
The Duke experience with 56 vulvar Paget disease patients was analyzed emphasizing pathologic features and controversial issues. Nearly all patients were Caucasian, and their mean age was 69 years. The average length of follow-up was 5.6 years. For each case, the following histologic features were evaluated and their association with disease course was examined: pseudo-invasion, adnexal involvement, signet-ring cells, cytologic atypia, glands formation, epidermal acantholysis, parakeratosis, hyperkeratosis, and chronic inflammation. The recurrence rate after surgical management was 32%, with epidermal acantholysis being the only statistically significant risk factor. Stromal invasion occurred in 10 patients (18%), and was not a statistically significant adverse prognostic indicator, although the single patient who died of the disease had the deepest stromal invasion. Recurrence was more common after resections with positive surgical margins, but this correlation was not statistically significant. Intraoperative frozen section analysis of the margins did not reduce recurrence rate, nor was it useful in attaining permanent free margins. The Paget cells were consistently reactive with cytokeratin-7 and carcinoembryonic antigen and unreactive with S-100 protein, HMB-45, and Mart-1. In addition, the tumor cells were usually positive for mucin stains. This profile helps distinguish vulvar Paget disease from its mimics, Pagetoid squamous cell carcinoma and malignant melanoma.
我们分析了杜克大学 56 例外阴派杰病患者的经验，强调了病理特征和有争议的问题。几乎所有患者均为白种人，平均年龄为 69 岁。平均随访时间为 5.6 年。对每个病例，评估了以下组织学特征，并检查了它们与疾病过程的关系：假浸润、附属物受累、印戒细胞、细胞学异型性、腺体形成、表皮棘层松解、角化过度、过度角化和慢性炎症。手术治疗后的复发率为 32%，表皮棘层松解是唯一具有统计学意义的危险因素。10 例（18%）患者发生间质浸润，但不是统计学上的不良预后指标，尽管唯一死于该病的患者有最深的间质浸润。切缘阳性的切除术后复发更为常见，但相关性无统计学意义。术中对切缘进行冷冻切片分析并未降低复发率，也无助于获得永久性无切缘。派杰细胞始终与细胞角蛋白 7 和癌胚抗原反应，与 S-100 蛋白、HMB-45 和 Mart-1 不反应。此外，肿瘤细胞通常对粘蛋白染色阳性。这种表型有助于将外阴派杰病与模仿物，如派杰样鳞状细胞癌和恶性黑色素瘤区分开来。
