关于非乳头的巴基特病（EMPD）的长期治疗效果和疾病特异性生存（DSS）的证据有限。为了评估大规模EMPD患者中手术和非手术治疗方法的DSS和治疗效果，我们进行了20家西班牙三级医疗机构的EMPD患者的回顾性病例研究。分析了249名患者的数据，其中中位随访时间为60个月。估计的5、10和15年DSS分别为95.9%、92.9%和88.5%。观察到深层真皮侵袭（≥1mm）或转移性疾病患者的DSS显著较低（p<0.05）。通过手术和局部治疗方法治疗的患者，EMPD病变大小达到了100%和75.3%的≥50%减小。广泛切除（WLE）后，42.4%的患者获得了无肿瘤切除边缘。莫氏镜手术（MMS）、具有无肿瘤切除边缘的WLE、具有阳性切缘的WLE、放射治疗和局部治疗后的5年无复发生存（RFS）分别为63.0%、51.4%、20.4%、30.1%和20.8%。回顾性设计。EMPD通常是一种良性预后的慢性疾病。MMS是该病效果最好的治疗选择。WLE后出现阳性切缘的复发率与使用局部药物治疗的患者相似。版权所有 © 2023 Elsevier Inc. 发布。

Evidence regarding long-term therapeutic outcomes and disease-specific survival (DSS) in Extramammary Paget's disease (EMPD) is limited.To assess the DSS and outcomes of surgical and nonsurgical therapeutic modalities in a large cohort of EMPD patients.Retrospective chart review of EMPD patients from 20 Spanish tertiary-care hospitals.Data on 249 patients with a median follow-up of 60 months were analyzed. The estimated 5, 10 and 15-year DSS was 95.9%, 92.9% and 88.5%, respectively. A significantly lower DSS was observed in patients showing deep dermal invasion (≥1mm) or metastatic disease (p<0.05). A ≥50% reduction in EMPD lesion size was achieved in 100% and 75.3% of patients treated with surgery and topical therapies, respectively. Tumor-free resection margins were obtained in 42.4% of the patients after wide local excision (WLE). The 5-year recurrence-free survival (RFS) after Mohs micrographic surgery (MMS), WLE with tumor-free margins, WLE with positive margins, radiotherapy and topical treatments was 63.0%, 51.4%, 20.4%, 30.1% and 20.8%, respectively.Retrospective design.EMPD is usually a chronic condition with favorable prognosis. MMS represents the therapeutic alternative with the greatest efficacy for the disease. Recurrence rates in patients with positive margins after WLE are similar to the ones observed in patients treated with topical agents.Copyright © 2023. Published by Elsevier Inc.