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

Leonor Fernandes; Joana Graça; Leonor Vasconcelos de Matos; Rita Sampaio; Mafalda Miranda Baleiras; Filipa Ferreira; Marta Mesquita Pinto; Helena Miranda; Ana Martins

doi:10.4081/dr.2020.8841

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

Dermatol Reports. 2020 Oct 22;12(2):8841. doi: 10.4081/dr.2020.8841. eCollection 2020 Sep 23.

Authors

Leonor Fernandes¹, Joana Graça¹, Leonor Vasconcelos de Matos¹, Rita Sampaio², Mafalda Miranda Baleiras¹, Filipa Ferreira¹, Marta Mesquita Pinto¹, Helena Miranda¹, Ana Martins¹

Affiliations

¹ Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental.
² Pathology Department, Centro Hospitalar Lisboa Ocidental, Lisboa, 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.

Keywords: Extramammary Paget Disease; Vulvar disease; brain metastasis; metastatic stage.