Department of Dermatology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea; Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
Department of Surgery, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
J Am Acad Dermatol. 2013 Apr;68(4):632-637. doi: 10.1016/j.jaad.2012.12.960. Epub 2013 Feb 8.
Extramammary Paget disease (EMPD) is a rare intraepithelial neoplasm of the skin characterized by ill-defined margins and high recurrence rates after wide local excision. Although Mohs micrographic surgery (MMS) has been proposed to decrease the rate of local recurrence, the efficacy of MMS for this condition has not yet been established.
We sought to assess the efficacy of MMS for the treatment of EMPD.
A comprehensive systematic review and individual patient data meta-analysis was performed including all available clinical studies and case reports with 5 or more subjects describing the use of MMS for EMPD.
Eight studies were identified and included in the current review: 3 retrospective studies and 5 case series. In all, 81 patients with 90 cases of MMS were included from these 8 studies. The overall recurrence rate for EMPD after MMS was 12.2% correlating with an estimated 5-year tumor-free rate of 83.6% by using Kaplan-Meier curve analysis. The treatment of EMPD with MMS resulted in significantly lower recurrence rates than wide local excision in this meta-analysis of 3 observational studies with comparators (odds ratio 0.20; 95% confidence interval 0.05-0.81).
Limitations include lack of controlled trials, small sample sizes in the included studies, and publication bias.
The current evidence supports the efficacy of MMS in the treatment of EMPD. Further controlled clinical trials are needed.
外阴派杰病（EMPD）是一种罕见的皮肤上皮内肿瘤，其特征为边界不清晰且经广泛局部切除后复发率较高。虽然已提出Mohs 显微外科手术（MMS）可降低局部复发率，但 MMS 对此病症的疗效尚未得到证实。
我们旨在评估 MMS 治疗 EMPD 的疗效。
进行了一项全面的系统评价和个体患者数据荟萃分析，包括所有可用的临床研究和 5 例或以上描述使用 MMS 治疗 EMPD 的病例报告。
共确定了 8 项研究并纳入本次综述：3 项回顾性研究和 5 项病例系列研究。总共纳入了这 8 项研究中的 81 名患者和 90 例 MMS 病例。通过 Kaplan-Meier 曲线分析，MMS 治疗 EMPD 的总复发率为 12.2%，估计 5 年无肿瘤生存率为 83.6%。对 3 项具有对照的观察性研究进行荟萃分析显示，MMS 治疗 EMPD 的复发率明显低于广泛局部切除（比值比 0.20；95%置信区间 0.05-0.81）。
局限性包括缺乏对照试验、纳入研究的样本量较小以及发表偏倚。
目前的证据支持 MMS 在 EMPD 治疗中的疗效。需要进一步开展对照临床试验。
