Metastatic extramammary paget disease, a remarkable clinical case and a brief review of a rare disease

  • Leonor Fernandes | fernandes.leonor@gmail.com Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal. https://orcid.org/0000-0001-6655-0986
  • Joana Graça Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal.
  • Leonor Vasconcelos de Matos Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal. https://orcid.org/0000-0001-9568-238X
  • Rita Sampaio Pathology Department, Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal.
  • Mafalda Miranda Baleiras Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal. https://orcid.org/0000-0003-2830-4287
  • Filipa Ferreira Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal. https://orcid.org/0000-0003-0083-4809
  • Marta Mesquita Pinto Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal. https://orcid.org/0000-0002-1052-3679
  • Helena Miranda Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal. https://orcid.org/0000-0002-2086-0193
  • Ana Martins Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental, Portugal.

Abstract

Extramammary Paget Disease (EMPD) is an often-misdiagnosed rare disorder, whose cause remains unknown. Diagnosis is confirmed by skin biopsy. Primary treatment for EMPD is surgery. Recurrence is common in the first two years and prognosis is good if the disease is localized and there is no underlying associated cancer. Patients with invasive and metastatic EMPD are uncommon and exhibit a poor prognosis, even when there is good response to a first chemotherapy line. Multiple chemotherapeutic regimens, with varying levels of success, have been attempted, but standard of care is not established. The central nervous system seems to be a common metastatic site with better survival than visceral metastasis.We report a case of metastatic EMPD that addresses the difficulties associated with the treatment of this rare disease, that has no current guidelines.

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Author Biography

Rita Sampaio, Pathology Department, Centro Hospitalar Lisboa Ocidental, Lisboa

 

 

References

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Published
2020-10-22
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Issue
Section
Case Reports
Keywords:
Extramammary Paget Disease, Vulvar disease, metastatic stage, brain metastasis
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How to Cite
Fernandes, L., Graça, J., Vasconcelos de Matos, L., Sampaio, R., Baleiras, M. M., Ferreira, F., Mesquita Pinto, M., Miranda, H., & Martins, A. (2020). Metastatic extramammary paget disease, a remarkable clinical case and a brief review of a rare disease. Dermatology Reports, 12(2). https://doi.org/10.4081/dr.2020.8841