Wilkinson Edward J, Brown Heather M
Department of Pathology, Immunology and Laboratory Medicine, University of Florida College of Medicine, Gainesville, FL 32610, USA.
Hum Pathol. 2002 May;33(5):549-54. doi: 10.1053/hupa.2002.124788.
Extramammary Paget disease is generally considered a distinct entity that can involve the genital tract skin and may be associated with underlying adenocarcinoma. Evidence is presented that vulvar Paget disease represents a heterogeneous group of epithelial neoplasms that can be similar both clinically and histopathologically. Three cases of vulvar Paget-like disease that were manifestations of urothelial carcinoma are investigated. Vulvar Paget disease can be classified based on the origin of the neoplastic Paget cells as either primary (of cutaneous origin) or secondary (of noncutaneous origin). Each classification has 3 subtypes: primary, intraepithelial cutaneous Paget disease of the usual type; intraepithelial cutaneous Paget disease with invasion, and intraepithelial cutaneous Paget disease as a manifestation of underlying skin appendage adenocarcinoma; secondary, Paget disease of anorectal origin, Paget disease of urothelial origin, and Paget disease of other origin. This subclassification is based on a review of the literature and the current study of 3 patients with Paget-like disease of urothelial neoplastic origin. The 3 subtypes of vulvar Paget disease studied here can present similarly as eczematoid skin or vulvar mucosal lesions and may appear similar on routine hematoxylin and eosin-stained slides. Immunohistochemical studies can be used to help differentiate them. The distinction between these 3 types of Paget-like lesions is essential in that the specific diagnosis has a significant influence on current treatment. The difference in surgical approach to the subtypes of vulvar Paget disease justifies classifying them into distinct lesions to avoid potential confusion and unnecessary surgery.
乳腺外佩吉特病通常被认为是一种独特的疾病实体，可累及生殖道皮肤，且可能与潜在的腺癌相关。有证据表明，外阴佩吉特病代表了一组异质性上皮性肿瘤，在临床和组织病理学上可能相似。本文对3例表现为尿路上皮癌的外阴佩吉特样疾病病例进行了研究。外阴佩吉特病可根据肿瘤性佩吉特细胞的起源分为原发性（皮肤源性）或继发性（非皮肤源性）。每种分类有3个亚型：原发性，通常类型的上皮内皮肤佩吉特病；伴有浸润的上皮内皮肤佩吉特病，以及作为潜在皮肤附属器腺癌表现的上皮内皮肤佩吉特病；继发性，肛门直肠源性佩吉特病、尿路上皮源性佩吉特病和其他源性佩吉特病。这种亚分类是基于对文献的回顾以及对3例尿路上皮肿瘤源性佩吉特样疾病患者的当前研究。本文研究的外阴佩吉特病的3个亚型在临床表现上可能类似湿疹样皮肤或外阴黏膜病变，在常规苏木精和伊红染色切片上可能看起来相似。免疫组织化学研究可用于帮助鉴别它们。这3种佩吉特样病变之间的区分至关重要，因为具体诊断对当前治疗有重大影响。外阴佩吉特病各亚型手术方法的差异证明将它们分类为不同的病变是合理的，以避免潜在的混淆和不必要的手术。
