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Anatomic Subtype Differences in Extramammary Paget Disease: A Meta-Analysis.
Nour Kibbi1, Joshua L Owen2,3, Brandon Worley4
1Department of Dermatology, Stanford University School of Medicine, Redwood City, California.
JAMA Dermatology
|March 6, 2024
Summary
Extramammary Paget disease (EMPD) diagnosis and treatment should vary by anatomic subtype. Vulvar EMPD may benefit from less invasive treatments, while perianal EMPD requires close surveillance for metastatic recurrence.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Extramammary Paget disease (EMPD) is a rare, recurrent skin cancer with unclear origins.
- The impact of anatomic location on EMPD presentation and management is not well understood.
Approach:
- Systematic review of MEDLINE, Embase, Web of Science, and Cochrane CENTRAL (1990-2022).
- Included studies reported on at least 3 patients, specified by anatomic subtype, and contained case-level data.
- Excluded non-English articles, studies with fewer than 3 patients, those lacking subtype data, and metastatic cases at presentation.
Key Points:
- Vulvar EMPD often presents asymptomatically, leading to delayed diagnosis and frequent intraepidermal disease, yet radical surgery is common, with a 34% recurrence rate.
- Penoscrotal EMPD has a lower recurrence rate (14%), but over a third of these recurrences involve regional or distant metastases.
- Perianal EMPD shows a 33.9% recurrence rate, with a significant proportion being regional or metastatic, and has the highest rate of invasive disease (50%).
Conclusions:
- Diagnosis and treatment strategies for EMPD should be tailored to the specific anatomic subtype.
- Consider less morbid treatments for primarily epidermal vulvar EMPD and enhance surveillance for local recurrence.
- Recommend close surveillance for metastatic recurrence in perianal EMPD and selective surveillance for the less common recurrences in penoscrotal EMPD.
