Metastatic Primary Extramammary Paget Disease: A Case Series

Serena Shimshak1, Hannah Berman1, Olayemi Sokumbi1

  • 1Department of Dermatology, Mayo Clinic, Jacksonville, Florida. Dr. Sokumbi also is from the Department of Laboratory Medicine and Pathology.

Cutis
|May 15, 2026
PubMed

Insights

Extramammary Paget disease (EMPD) can rapidly progress to fatal metastasis, even without other cancers. This review highlights aggressive invasive EMPD cases and emphasizes prompt management for better outcomes.

Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Extramammary Paget disease (EMPD) is a rare skin cancer.
  • It often presents as itchy, red patches in apocrine gland areas.
  • Dermal invasion correlates with metastasis and poor prognosis.

Purpose of the Study:

  • To present two cases of invasive EMPD with rapid progression.
  • To review literature on clinicopathologic features, management, and prognosis.
  • To underscore the aggressive potential of invasive EMPD.

Main Methods:

  • Case report presentation.
  • Literature review of invasive EMPD.
  • Analysis of clinicopathologic features and outcomes.

Main Results:

  • Two patients with invasive EMPD experienced rapid disease progression and died from metastasis.
  • No other underlying malignancies were found in these cases.
  • Literature review confirmed dermal invasion as a critical prognostic factor.

Conclusions:

  • Invasive EMPD can exhibit rapid, fatal metastatic progression.
  • Early recognition and management are crucial.
  • Further research into aggressive EMPD subtypes is warranted.