Narayan Vikram M, Master Viraj A
Department of Urology, Emory University School of Medicine, Atlanta, Georgia, USA.
Curr Opin Urol. 2022 Mar 1;32(2):211-215. doi: 10.1097/MOU.0000000000000948.
Extramammary Paget's Disease (EMPD) is an uncommon intra-epithelial malignancy, affecting primarily apocrine gland-bearing skin. EMPD is often considered an orphan diagnosis given its rarity. This review provides a contemporary overview of EMPD management.
The mainstay of EMPD treatment centers around a high index of suspicion to allow for an early and accurate diagnosis, wide local or Mohs micrographic surgical excision with care paid toward the margin status, and thoughtful consideration for lymphadenectomy in patients with clinically positive regional disease. There is currently no consensus regarding adjuvant therapies or systemic therapies although with ongoing improvements in tumor biology and genomics, including molecular pathways and alterations specific to EMPD, targeted or combinatorial therapies may be on the horizon.
Clinicians caring for patients with EMPD should seek consultation from or if feasible, consider referral to high-volume, experienced centers with patients counseled and provided with frequent and close follow-up for disease recurrence or progression. Collaboration with groups such as the Global Society for Rare Genitourinary Tumors, and especially patient groups will be vital to designing trials and collaborative databases.
乳腺外佩吉特病（EMPD）是一种罕见的上皮内恶性肿瘤，主要累及含顶泌汗腺的皮肤。鉴于其罕见性，EMPD常被视为一种罕见病诊断。本综述提供了EMPD治疗的当代概述。
EMPD治疗的主要方法包括高度怀疑以实现早期准确诊断、广泛局部切除或莫氏显微外科手术切除并关注切缘状态，以及对临床区域病变阳性患者进行淋巴结清扫的慎重考虑。目前关于辅助治疗或全身治疗尚无共识，尽管随着肿瘤生物学和基因组学的不断进步，包括EMPD特有的分子途径和改变，靶向治疗或联合治疗可能即将出现。
诊治EMPD患者的临床医生应寻求会诊，或在可行的情况下，考虑转诊至高容量、经验丰富的中心，并为患者提供咨询，对疾病复发或进展进行频繁密切的随访。与全球罕见泌尿生殖系统肿瘤协会等组织合作，尤其是与患者组织合作，对于设计试验和协作数据库至关重要。
