文章：

从诊断到治疗：原发性皮肤外乳腺佩吉特病的非侵入性与非手术方法系统综述

From Diagnosis to Therapy in Primary Cutaneous Extramammary Paget’s Disease: A Systematic Review of Non-Invasive and Non-Surgical Approaches

原文发布日期：6 November 2025

DOI: 10.3390/cancers17213594

类型: Article

开放获取: 是

英文摘要：

Background/Objectives: Extramammary Paget’s disease (EMPD) is a rare cutaneous malignancy arising in areas rich in apocrine glands that poses diagnostic and therapeutic difficulties. Although surgery remains the standard of care, achieving clear margins is challenging and recurrence rates are high. This review explores the contribution of non-invasive imaging for diagnosis and monitoring, and evaluates conservative, non-surgical therapies as alternatives to radical surgery.Methods: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), a systematic review was conducted: eligible studies included interventional and observational research, as well as case series and reports, assessing non-invasive diagnostic methods or non-surgical treatments for EMPD. Data extraction and risk-of-bias evaluation were performed independently by multiple reviewers, and a narrative synthesis summarized therapeutic outcomes and diagnostic performance.Results: Of 808 identified records, 82 met the inclusion criteria: 66 focused on non-surgical therapies, 15 on diagnostic techniques, and one on both. Reflectance confocal microscopy (RCM) and photodynamic diagnosis (PDD) showed high concordance with histopathology, aiding both diagnosis and margin delineation. Among therapies, topical imiquimod and photodynamic therapy (PDT) demonstrated encouraging response rates, while radiotherapy, laser ablation, and systemic chemotherapy were less consistently reported. Evidence quality was limited by small cohorts, heterogeneous regimens, and variable follow-up.Conclusions: Non-invasive imaging enhances diagnostic accuracy and surgical planning, while non-surgical treatments—particularly imiquimod and PDT—offer viable alternatives in selected cases. Larger prospective studies are needed to establish standardized protocols and clarify long-term outcomes.

摘要翻译： 

背景/目的：乳房外佩吉特病（EMPD）是一种罕见的皮肤恶性肿瘤，好发于富含顶泌汗腺的区域，其诊断与治疗均存在困难。虽然手术仍是标准治疗方式，但实现切缘阴性具有挑战性且复发率高。本综述探讨无创影像学在诊断与监测中的作用，并评估可作为根治性手术替代方案的保守性非手术治疗方法。 方法：遵循系统综述和荟萃分析优先报告条目（PRISMA）指南，我们进行了系统性文献回顾：纳入研究包括评估EMPD无创诊断方法或非手术治疗的干预性及观察性研究、病例系列及病例报告。数据提取与偏倚风险评估由多位研究者独立完成，并通过叙述性综合总结治疗效果与诊断效能。 结果：在808篇初筛文献中，82篇符合纳入标准：其中66篇聚焦非手术治疗，15篇关注诊断技术，1篇同时涉及两方面。反射式共聚焦显微镜（RCM）与光动力诊断（PDD）显示与组织病理学高度一致，有助于诊断及切缘界定。在治疗方面，局部使用咪喹莫特与光动力疗法（PDT）显示出令人鼓舞的应答率，而放射治疗、激光消融及全身化疗的相关报道则一致性较低。现有证据质量受限于样本量小、治疗方案异质性及随访时间差异等因素。 结论：无创影像学技术可提升诊断准确性并优化手术规划，而非手术治疗——尤其是咪喹莫特与PDT——为特定病例提供了可行的替代方案。未来需要更大规模的前瞻性研究以建立标准化治疗方案并明确长期预后。

原文链接：

From Diagnosis to Therapy in Primary Cutaneous Extramammary Paget’s Disease: A Systematic Review of Non-Invasive and Non-Surgical Approaches