Background: Extramammary Paget’s disease (EMPD) is a rare cutaneous carcinoma that typically affects the elderly and is frequently observed in genital and perianal regions. We analyzed the outcomes and prognostic factors for EMPD after radiation therapy (RT).Methods: We analyzed data from 81 patients with non-metastatic EMPD who received either RT alone or in combination with surgery and/or chemotherapy. The median radiation dose was 56 Gy in 28 fractions. Local control (LC), progression-free survival (PFS), and overall survival (OS) rates were calculated using the Kaplan–Meier method. Multivariate analyses were performed using the Cox proportional hazards model. Late adverse events were evaluated by NCI-CTCAE version 5.0.Results: The median age was 78 years, and the median follow-up period was 36 months. The three-year LC, PFS, and OS rates were 75%, 52%, and 80%, respectively. Multivariate analyses identified the presence of lymph node (LN) metastasis, the absence of surgery, and the omission of elective nodal irradiation (i.e., local irradiation only) as significant factors for unfavorable LC (p= 0.01, 0.02, and 0.006) and PFS (p= 0.001, 0.04, and 0.03). LN metastasis was also a significant factor for unfavorable OS (p= 0.005). One patient developed grade 2 skin infection, and another developed grade 3 lymphedema; no grade 4 or higher toxicity was observed.Conclusions: The present results revealed prognostic factors for EMPD after RT and suggest that the absence of surgery and omission of elective nodal irradiation worsened outcomes. A prospective study is needed to establish an optimal treatment strategy for this rare malignancy, which is common in the elderly.
背景：乳房外佩吉特病（EMPD）是一种罕见的皮肤癌，通常影响老年人，常见于生殖器和肛周区域。本研究分析了EMPD患者接受放射治疗（RT）后的疗效及预后因素。 方法：我们分析了81例非转移性EMPD患者的数据，这些患者接受了单纯放疗或联合手术和/或化疗。中位放疗剂量为56 Gy，分28次完成。采用Kaplan-Meier法计算局部控制率（LC）、无进展生存率（PFS）和总生存率（OS）。采用Cox比例风险模型进行多因素分析。晚期不良事件依据NCI-CTCAE 5.0版标准进行评估。 结果：患者中位年龄为78岁，中位随访时间为36个月。三年LC率、PFS率和OS率分别为75%、52%和80%。多因素分析显示，淋巴结（LN）转移、未接受手术以及未行选择性淋巴结照射（即仅行局部照射）是LC不良（p值分别为0.01、0.02和0.006）和PFS不良（p值分别为0.001、0.04和0.03）的显著影响因素。淋巴结转移也是OS不良的显著因素（p=0.005）。1例患者出现2级皮肤感染，另1例出现3级淋巴水肿；未观察到4级或更高级别的毒性反应。 结论：本研究结果揭示了EMPD放疗后的预后因素，并表明未接受手术和未行选择性淋巴结照射会恶化治疗结局。需要开展前瞻性研究，为这种在老年人中常见的罕见恶性肿瘤制定最佳治疗策略。
肿瘤(癌症)患者之家