Int J Gynecol Cancer. 2018 May;28(4):829-839. doi: 10.1097/IGC.0000000000001237.
OBJECTIVE/PURPOSE: Extramammary Paget disease (EMPD) is a rare neoplasm of the skin generally affecting the anogenital area. Because of the low-frequency of the disease, no specific guidelines about the treatment strategy are available. Surgery is the recommended therapy for resectable and localized disease, but several other local treatments have been reported such as radiotherapy (RT). Most articles report small retrospective studies, referring to patients treated decades ago with large heterogeneity in terms of RT dose and technique. The aim of this study was to systematically review the main experiences in RT for the treatment of EMPD in the past 30 years.
A systematic search of the bibliographic databases PubMed and Scopus from January 1986 to January 2017 was performed including studies published in English, Italian, Spanish, French, and German language.
According to the search strategy, 19 full-text articles, published from 1991 to 2015, fulfilled inclusion criteria and were included in the final review. All articles were retrospective analyses with no randomized controlled trials. These studies evaluated 195 EMPD patients treated with RT, delivered in several settings. A large variability in terms of RT doses, fractionation, clinical setting, and techniques was found.Radiotherapy was administered as definitive treatment for primary or recurrent disease after surgery in 18 studies with doses ranging from 30 to 80.2 Gy delivered in 3 to 43 fractions. Radiotherapy was administered as postoperative adjuvant treatment in 9 articles with doses ranging between 32 and 64.8 Gy in 20 to 30 fractions. Two studies reported the RT use in preoperative neoadjuvant setting with doses ranging between 40 and 43.30 Gy, and 2 experiences reported the RT treatment for in situ EMPD, using 39.6 to 40 Gy. Adverse events were reported in almost all but 2 articles and were grade 2 or lower.The 18 studies evaluating RT as definitive treatment for primary or recurrent disease after surgery reported a complete response rate ranging from 50% to 100%, with a variable rate of local relapse or persistent disease ranging from 0% to 80% of cases. The 9 studies evaluating RT as postoperative adjuvant treatment reported a local relapse or persistent disease rate of 0% to 62.5%. A dose-response relationship was reported suggesting doses greater than or equal to 60 Gy for gross tumor volume treatment. Local control, disease-free survival, and overall survival at 12, 20, and 60 months have been retrieved for available data, respectively.In patients with EMPD and concurrent underlying internal malignancy, the prognosis was often worsened by the latter. In this setting, literature analysis showed a potential RT palliative role for symptoms control or local control maintenance.Derma tumor invasion greater than 1 mm and lymph node metastases were reported to be important prognostic factors for distant metastases or death.
To date, literature highlights the role of RT in the management of EMPD, but with low level of evidences.
目的/目的：外阴派杰病（EMPD）是一种罕见的皮肤肿瘤，通常影响肛门生殖器区域。由于疾病的低频率，没有关于治疗策略的具体指南。手术是可切除和局部疾病的推荐治疗方法，但也有其他几种局部治疗方法，如放射治疗（RT）。大多数文章报告了小的回顾性研究，涉及几十年前用大异质性 RT 剂量和技术治疗的患者。本研究的目的是系统地回顾过去 30 年 RT 治疗 EMPD 的主要经验。
对 1986 年 1 月至 2017 年 1 月的文献数据库 PubMed 和 Scopus 进行了系统搜索，包括以英语、意大利语、西班牙语、法语和德语发表的研究。
根据搜索策略，有 19 篇全文文章，发表于 1991 年至 2015 年，符合纳入标准，并纳入最终审查。所有文章均为回顾性分析，无随机对照试验。这些研究评估了 195 名接受 RT 治疗的 EMPD 患者，这些患者在不同的环境中接受了治疗。在 RT 剂量、分割、临床环境和技术方面存在很大的差异。放射治疗在 18 项研究中作为手术后原发性或复发性疾病的根治性治疗，剂量范围为 30 至 80.2 Gy，分为 3 至 43 个剂量。在 9 项研究中，放射治疗作为术后辅助治疗，剂量范围为 32 至 64.8 Gy，分为 20 至 30 个剂量。两项研究报告了在术前新辅助治疗中使用 40 至 43.30 Gy 的 RT，两项经验报告了用于原位 EMPD 的 RT 治疗，使用 39.6 至 40 Gy。几乎所有文章都报告了不良反应，但有 2 篇文章没有报告，不良反应为 2 级或更低。评估 RT 作为手术后原发性或复发性疾病的根治性治疗的 18 项研究报告了完全缓解率为 50%至 100%，局部复发或持续性疾病的发生率为 0%至 80%。评估 RT 作为术后辅助治疗的 9 项研究报告了局部复发或持续性疾病的发生率为 0%至 62.5%。报告了剂量反应关系，提示肿瘤体积治疗的剂量大于或等于 60 Gy。对于可获得的数据，分别获得了 12、20 和 60 个月的局部控制、无病生存和总生存的局部控制。对于患有 EMPD 和并发内部恶性肿瘤的患者，后者常使预后恶化。在这种情况下，文献分析表明 RT 具有缓解症状或维持局部控制的姑息作用。
皮肤肿瘤侵犯>1 毫米和淋巴结转移被报道为远处转移或死亡的重要预后因素。
迄今为止，文献强调了 RT 在 EMPD 管理中的作用，但证据水平较低。
