乳房外佩吉特病 (EMPD) 可能与潜在的内部腺癌相关，称为继发性 EMPD。 EMPD 解剖亚型之间的这种关联的差异以及对筛查的影响尚未完全理解。根据 EMPD 解剖亚型定义继发性 EMPD 的发生率和相关腺癌的类型，并提出潜在腺癌的筛查算法。EMPD 的系统文献综述（1990 年 1 月- 2022 年 11 月）。一百二十二项研究符合纳入标准。多学科专家小组审查了有关腺癌筛查的建议声明。与阴茎阴囊和外阴 EMPD（各 6%，主要源自泌尿生殖系统）相比，肛周 EMPD 与潜在腺癌（25%，主要是结直肠癌）的高发生率相关。肛周 EMPD 的彻底筛查包括结肠镜检查、尿液细胞学检查以及胸部、腹部和骨盆的计算机断层扫描 (CT)。低风险阴茎阴囊疾病的成本意识筛查测试包括尿细胞学检查、血红素隐匿测试和前列腺特异性抗原测试（尤其是 70 岁以下的患者）。对于低风险外阴 EMPD，建议进行尿细胞学检查和乳房 X 光检查。具有高风险特征的 EMPD 可能需要更敏感的器官特异性测试。选择偏倚；没有系统随访的回顾性数据。EMPD 中潜在腺癌的筛查应以解剖位置为指导。版权所有 © 2024。由 Elsevier Inc. 出版。

Extramammary Paget's disease (EMPD) may be associated with an underlying internal adenocarcinoma, referred to as secondary EMPD. Differences in this association by EMPD anatomic subtype and implications for screening are not fully understood.Define the rates of secondary EMPD and types of associated adenocarcinomas by EMPD anatomic subtype and propose a screening algorithm for underlying adenocarcinoma.Systematic literature review of EMPD (January 1990- November 2022). One hundred twenty-two studies met the inclusion criteria. A multidisciplinary expert panel reviewed the recommendation statements on adenocarcinoma screening.Perianal EMPD was associated with a high rate of underlying adenocarcinoma (25%, primarily colorectal) compared with penoscrotal and vulvar EMPD (6% each, primarily of genitourinary origin). Thorough screening in perianal EMPD includes a colonoscopy, urine cytology, and computed tomography (CT) of the chest, abdomen and pelvis. Cost-conscious screening tests in low-risk penoscrotal disease include urine cytology, heme-occult test, and prostate-specific antigen test (especially if under 70 years of age). For low-risk vulvar EMPD, urine cytology and mammography are recommended. EMPD with high-risk features may warrant more sensitive organ-specific testing.Selection bias; retrospective data without systematic follow-up.Screening for underlying adenocarcinoma in EMPD should be guided by anatomic location.Copyright © 2024. Published by Elsevier Inc.