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Extramammary Paget's Disease.

Mackenzie Asel¹, Nicole R LeBoeuf²

¹Department of Dermatology, The Centers for Melanoma and Cutaneous Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02115, USA.

Hematology/Oncology Clinics of North America

|December 1, 2018

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Extramammary Paget disease (EMPD) is a rare skin cancer impacting quality of life. This review covers workup and management options for EMPD, noting its potential association with other cancers.

Area of Science:

Dermatology
Oncology

Background:

Extramammary Paget disease (EMPD) is a rare malignancy primarily affecting the elderly in the genital, perineal, and perianal regions.
While often confined to the epidermis, EMPD can become invasive, potentially spreading to adjacent tissues or indicating distant synchronous malignancies.

Purpose of the Study:

To review the diagnostic workup and management strategies for patients diagnosed with Extramammary Paget disease.
To address the lack of evidence-based treatment guidelines and highlight the importance of comprehensive evaluation due to potential cancer associations.

Main Methods:

Literature review of existing studies and clinical guidelines on EMPD.
Analysis of diagnostic approaches, including imaging and biopsy.
Evaluation of various treatment modalities and their outcomes.

Keywords:

Diagnosis EMPD Extramammary Paget's disease Management options Prognosis

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Main Results:

EMPD diagnosis requires thorough evaluation due to its potential for invasion and association with other cancers.
Treatment approaches for EMPD are diverse, with limited evidence-based recommendations.
Management strategies must consider the extent of the disease and potential underlying or synchronous malignancies.

Conclusions:

Formal evaluation is crucial for all patients with EMPD to rule out invasive disease and other cancers.
Standardized, evidence-based treatment protocols for EMPD are needed.
Multidisciplinary management may be beneficial for optimizing patient outcomes.