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Published on: July 29, 2017
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.
Abstract:
Extramammary Paget disease (EMPD) is an uncommon cutaneous malignancy that manifests with pruritic erythematous plaques within apocrine-rich areas such as the axillae and anogenital region. Dermal invasion is a known risk factor for metastasis, which is associated with poor outcomes. We present 2 cases of invasive EMPD on initial biopsy with rapid disease progression. The patients died secondary to metastatic EMPD without additional underlying malignancy. We review the literature and highlight key clinicopathologic features, management considerations, and the potential for rapid disease progression in cases of invasive EMPD.
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.
