A Primer on Extramammary Paget's Disease for the Urologist

Joon Yau Leong; Paul H. Chung

Disclosures

Transl Androl Urol. 2020;9(1):93-105. 

In This Article

Abstract and Introduction

Abstract

Extramammary Paget's disease (EMPD) is a rare and lethal intraepithelial malignancy that remains poorly understood. No standardized guidelines or consensus statements exist with regards to the diagnostic evaluation, therapeutic approaches and follow-up management. Complete surgical excision with negative margins has been accepted as the mainstay of treatment for EMPD to decrease the risk of local recurrence and to maximize durable cure. Non-invasive therapies, such as laser therapy, photodynamic therapy, radiotherapy and topical chemotherapy have been utilized, but are best reserved for non-surgical candidates. While the debate on the surgical approach between wide local excision (WLE) and Mohs micrographic surgery (MMS) continues, several studies have demonstrated the ability of WLE to be performed safely and effectively and to yield equally satisfactory outcomes with similar rates of recurrence to MMS. Patients undergoing surgical excision often require complex closures with skin grafting or local flaps to close genital defects. We aim to provide an up-to-date review of the current knowledge of EMPD. In addition to discussing the clinical presentation and prognostic outcomes, we focus and elaborate on the diagnostic approaches and treatment alternatives available. This information may serve as a primer for the urologist who may be called upon to treat this malignancy primarily or to provide wound coverage secondarily.

Introduction

Extramammary Paget's disease (EMPD) is a rare yet lethal cutaneous malignancy with an overall survival rate of 60.2% at 10 years postdiagnosis.[1] This disease is distinct from Paget's disease of the breast and predominantly affects the apocrine gland-bearing skin such as the perianal, genital and axillary regions of the body. Due to the rarity of this disease, controversies exist within the literature regarding its true prevalence, its association with concurrent internal malignancies and the diagnostic evaluation, therapeutic approaches and follow-up management. Currently, treatment recommendations for EMPD are based off single institution series and small case reports only, with many different treatment options, both in clinical and experimental use, yielding variable outcomes and results.[2–4] We aim to provide an up-to-date review of the current knowledge of EMPD. In addition to discussing the clinical presentation and prognostic outcomes of this disease, we also focus and elaborate on the diagnostic approaches and treatment alternatives available to physicians when faced with patients presenting with this malignancy.

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