Department of Dermatology, Toyama Prefectural Central Hospital, Toyama, Japan.
Department of Dermatology, National Cancer Center Hospital, Tokyo, Japan.
Exp Dermatol. 2024 Feb;33(2):e15030. doi: 10.1111/exd.15030.
Information about extramammary Paget's (EMPD) treatment is limited because of the rarity of the disease. The prognosis differs between in situ EMPD and invasive EMPD; therefore, therapy should be planned according to the disease stage. We collected data on 643 EMPD cases treated between 2015 and 2019 in Japan and assessed recent trends in EMPD treatment and prognosis based on the EMPD-oriented TNM staging. Among the 643 patients, 317 had stage 0 (49.3%), 185 had stage I (28.8%), 51 had stage II (7.9%), 18 had stage IIIA (2.8%), 48 had stage IIIB (7.5%) and 24 had stage IV (3.7%) disease. Each stage showed a distinct survival curve, with the exception of stages II and IIIA. Curative surgery was most common in patients with stage 0-III disease. Chemotherapy was the first-line therapy, mainly in patients with stage IIIB and IV disease, most commonly with docetaxel (DTX), followed by DTX + tegafur gimeracil oteracil potassium (TS-1) and TS-1. Patients with local disease exhibited a 4.4% recurrence rate. Univariate analysis revealed no prognostic differences according to age, sex or primary tumour site. SLNB was not related to disease-specific survival. In multivariate analysis, female sex significantly predicted local relapse in stage 0-I (HR 3.09; 95% CI, 1.13-8.43), and initial treatment with curative surgery was significantly protective in terms of disease-specific survival in stage II-IIIA (HR, 0.17; 95% CI, 0.04-0.71) and stage IIIB-IV (HR 0.16; 95% CI, 0.05-0.51). Further clinical studies are needed to improve the prognosis of patients with stage II-IV EMPD.
有关乳房外派杰氏病（EMPD）治疗的信息有限，因为该病的发病率较低。原位 EMPD 和侵袭性 EMPD 的预后不同；因此，应根据疾病阶段制定治疗方案。我们收集了 2015 年至 2019 年在日本治疗的 643 例 EMPD 病例的数据，并根据 EMPD 定向 TNM 分期评估了 EMPD 治疗和预后的近期趋势。在 643 例患者中，有 317 例为 0 期（49.3%），185 例为 1 期（28.8%），51 例为 2 期（7.9%），18 例为 3A 期（2.8%），48 例为 3B 期（7.5%），24 例为 4 期（3.7%）。每个阶段都显示出明显的生存曲线，除了 2 期和 3A 期。0-3 期疾病患者最常接受根治性手术。化疗是一线治疗，主要用于 3B 期和 4 期疾病患者，最常用的药物是多西紫杉醇（DTX），其次是 DTX+替加氟吉美嘧啶奥替拉西钾（TS-1）和 TS-1。局部疾病患者的复发率为 4.4%。单因素分析显示，年龄、性别或原发肿瘤部位与预后无关。SLNB 与疾病特异性生存无关。多因素分析显示，0-1 期女性患者局部复发的风险显著升高（HR 3.09；95%CI，1.13-8.43），2-3A 期初始根治性手术治疗显著降低疾病特异性生存风险（HR 0.17；95%CI，0.04-0.71）和 3B-4 期（HR 0.16；95%CI，0.05-0.51）。需要进一步的临床研究来改善 2-4 期 EMPD 患者的预后。
