Padrnos Leslie, Karlin Nina, Halfdanarson Thorvardur R
Hematology Oncology Department, Mayo Clinic Arizona , Phoenix, AZ.
Hematology Oncology Department, Mayo Clinic Rochester , MN, USA.
Rare Tumors. 2016 Nov 17;8(4):6804. doi: 10.4081/rt.2016.6804.
Extramammary Paget disease (EMPD) is a rare cutaneous malignancy. The most common presentation of EMPD is the vulva followed by perianal involvement. Most cases are localized to the dermis with treatment focused on surgery, topical treatment or radiotherapy. Recurrence is frequent despite therapies utilized. Metastatic extramammary Paget disease is uncommon and, as such, standard treatment guidelines do not exist. This study sought to evaluate the treatment regimens and outcomes of patients treated at a Mayo Clinic Center from 1998-2012. Cancer registry inquiry revealed 261 patients with report advanced Paget disease during these years. Ten cases of metastatic EPMD were identified with sufficient documentation for review. This review reveals support for utilizing localized radiation therapy for bulky disease sequentially with systemic chemotherapy consisting of carboplatin and paclitaxel or irinotecan. Further studies are necessary to define the optimal treatment regimen.
乳房外佩吉特病（EMPD）是一种罕见的皮肤恶性肿瘤。EMPD最常见的表现部位是外阴，其次是肛周受累。大多数病例局限于真皮层，治疗重点是手术、局部治疗或放疗。尽管采用了各种治疗方法，但复发很常见。转移性乳房外佩吉特病并不常见，因此不存在标准的治疗指南。本研究旨在评估1998年至2012年在梅奥诊所中心接受治疗的患者的治疗方案和治疗结果。癌症登记查询显示，这些年有261例报告患有晚期佩吉特病的患者。确定了10例有充分记录可供审查的转移性EMPD病例。该综述表明，对于体积较大的疾病，支持先采用局部放射治疗，随后采用由卡铂和紫杉醇或伊立替康组成的全身化疗。需要进一步研究来确定最佳治疗方案。
