Open Access

REVIEW

Extramammary Paget’s disease: what do we know and how do we treat?

Jacob J. Adashek¹, Alex Leonard^2,*, Samantha W. Nealon³, Arvind Krishnan³, Gerard C. Mosiello⁴, Jasreman Dhillon⁵, Philippe E. Spiess³

1 Department of Internal Medicine, University of South Florida, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA
2 Morsani College of Medicine, University of South Florida, Tampa, Florida, USA
3 Department of Genitourinary Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA
4 Department of Cutaneous Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA
5 Department of Anatomical Pathology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA
* authors contributed equally
Address correspondence to Dr. Philippe E. Spiess, Department of Genitourinary Oncology, Moffitt Cancer Center, Department of Urology, 12902 USF Magnolia Drive, Tampa, FL 33612-9416 USA

Canadian Journal of Urology 2019, 26(6), 10012-10021.

Abstract

Introduction: Extramammary Paget’s disease (EMPD) is a rare and complex condition, for which no established guidelines exist regarding diagnosis and management. There have been recent improvements in the diagnosis and management of EMPD, largely due to an enhanced understanding of its underlying pathogenesis.
Materials and methods: A literature search on PubMed including articles that describe pathogenesis, clinical diagnosis, treatment modalities, and future treatment were selected and included to build this review.
Results: Recent studies suggest the expression of HER2 and androgen receptors, which could be useful targets for future treatment strategies. Carcinoembryonic antigen as a biomarker for EMPD has shown the potential to aid in the detection of metastatic EMPD and assessment of treatment response. Studies have also demonstrated that the initial site of EMPD can be predictive of secondary malignancies, which helps guide initial workup and evaluation.
Conclusions: Significant developments in understanding the pathogenesis of EMPD have been made, especially regarding the genomic aberrations associated with EMPD. This has allowed for the development and use of therapeutic options that may improve outcomes for patients with EMPD.

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