Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Mar;46(3):305-311.
doi: 10.1097/DSS.0000000000002240.

Extramammary Paget's Disease: A Review of the Literature Part II: Treatment and Prognosis

Caroline R Morris  1 , Eva A Hurst
Affiliations
Review

Extramammary Paget's Disease: A Review of the Literature Part II: Treatment and Prognosis

Caroline R Morris et al. Dermatol Surg. 2020 Mar.

Abstract

Background: Extramammary Paget's disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine-rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence.

Objective: To review the current literature on EMPD regarding treatment and prognosis.

Materials and methods: Literature review using PubMed search for articles related to EMPD.

Results: Extramammary Paget's disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy.

Conclusion: Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%.

PubMed Disclaimer

References

    1. Hatta N, Yamada M, Hirano T, Fujimoto A, et al. Extramammary Paget's disease: treatment, prognostic factors and outcome in 76 patients. Br J Dermatol 2008;158:313–8.
    1. Marchesa P, Fazio V, Oliart S, Goldblum JR, et al. Long-term outcome of patients with perianal Paget's disease. Ann Surg Onc 1997;4:475–80.
    1. Lam C, Funaro D. Extramammary Paget's disease: summary of current knowledge. Dermatol Clin 2010;28:807–26.
    1. Abide JA, Nahai F, Benett RG. The meaning of surgical margins. Plast Reconstr Surg 1984;73:492–7.
    1. Zollo JD, Zeitouni NC. The roswell park cancer institute experience with extramammary Paget's disease. Br J Dermatol 2000;142:59–65.