Extramammary Paget’s disease: Updates in the workup and management

作     者：Reza Nabavizadeh Khushali B.Vashi Behnam Nabavizadeh Vikram M.Narayan Viraj A.Master Reza Nabavizadeh;Khushali B.Vashi;Behnam Nabavizadeh;Vikram M.Narayan;Viraj A.Master

作者机构：Department of UrologyEmory University School of MedicineAtlantaGAUSA Department of UrologyWeill Cornell MedicineNew YorkNYUSA Winship Cancer InstituteAtlantaGAUSA 

出 版 物：《Asian Journal of Urology》 (亚洲泌尿外科杂志（英文）)

年 卷 期：2022年第9卷第4期

页      面：451-459页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：Extramammary Paget’s disease Extramammary Paget’s malignancy Genitourinary Paget’s disease Rare genitourinary tumors 

摘      要：Objective:Extramammary Paget’s disease(EMPD)is a rare cutaneous malignant *** to its rarity,there is a paucity of data regarding best treatment *** primarily affects apocrine gland-bearing skin areas such as the vulva,scrotum,and *** objective was to provide a present-day rationale for diagnosis,pathogenesis,and treatment of EMPD with a focus on recent progress in workup and management of the ***:Literature on EMPD until February 2022 was assessed through PubMed,MEDLINE databases,and Google scholar.A narrative review of the most relevant articles was ***:EMPD usually presents with indolent growth while usually being diagnosed primarily as carcinoma in *** foundation of EMPD treatment centers around prompt and accurate diagnosis,wide local or Mohs micrographic surgical excision with proper management towards the margin status,and careful consideration for lymphadenectomy in patients with regionally positive *** chemotherapies are alternative treatments modality for patients with distant metastases;however,they sometimes have suboptimal *** present,there is no agreement regarding adjuvant or systemic therapies,although recent studies have shown several insights into the molecular pathogenesis,tumor biology,and genomics of the development and advancement of EMPD,which may lead to novel and targeted treatment approaches for metastatic EMPD in the ***:Patients with EMPD should seek care from physicians with expertise in disease management and patient *** patients should be surveilled with close follow-up to evaluate them for disease recurrence or *** collaborations with groups such as the Global Society for Rare Genitourinary Tumors,and especially patient support groups are crucial in designing clinical trials to help elucidate more robust data in this orphan disease.