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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
Summary
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.
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.