Introduction

The complexity of Extramammary Paget’s Disease (EMPD) has been apparent for decades with minimal improvements in diagnostic and therapeutic options. This rare carcinoma generally afflicts individuals greater than 60 years old and more often Caucasians than any other ethnicity.1 There are a multitude of case series, case reports, and retrospective studies, offering various treatment protocols; however, there is insufficient evidence for clear management guidelines. The heterogeneity of this disease in its presentation, location, depth of invasion and its typical multidisciplinary approach to management make it difficult to treat. The association with other malignancies is a well-described phenomenon and should inform treatment and long term management.2 Use of biomarkers shows promise in diagnosis and treatment monitoring. As immunotherapy (IO) is becoming a mainstay for many cancers, there is growing support for the use of these agents in advanced EMPD patients.

There have been significant developments in understanding the pathogenesis of EMPD which have allowed for noteworthy improvements in its diagnosis and management. In particular, a better understanding of the genomic aberrations associated with EMPD has allowed for the development and use of therapeutic options which may improve outcomes for patients with EMPD. Furthermore, the use of biomarkers has the potential to enhance the ability to detect disease earlier and monitor response to treatment. While EMPD lesions biopsy is pivotal in determining surgical margins, no standardized template has been produced. However, development of standardized templates, such as the one used at Moffitt Cancer Center, have the potential to improve the initial diagnostic evaluation of EMPD.

At this time, there are no established guidelines regarding treatment modalities for EMPD. There are various treatment options for localized EMPD; however, less is known about the best treatment modalities for metastatic disease. Neoadjuvant or adjuvant therapy with topical imiquimod or radiation therapy, in addition to surgical resection, may be beneficial for patients with localized disease. For metastatic disease, there is a paucity of evidence to make recommendations for systemic therapy and randomized controlled trials are imperative in order to determine how to provide optimal care and treatment guideline for these patients.