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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.

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|March 6, 2024
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Summary
This summary is machine-generated.

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.

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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.