Fernandes Leonor, Graça Joana, de Matos Leonor Vasconcelos, Sampaio Rita, Baleiras Mafalda Miranda, Ferreira Filipa, Pinto Marta Mesquita, Miranda Helena, Martins Ana
Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental.
Pathology Department, Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal.
Dermatol Reports. 2020 Oct 22;12(2):8841. doi: 10.4081/dr.2020.8841. eCollection 2020 Sep 23.
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.
乳房外佩吉特病（EMPD）是一种常被误诊的罕见疾病，其病因尚不清楚。通过皮肤活检确诊。EMPD的主要治疗方法是手术。复发在前两年很常见，如果疾病局限且无潜在相关癌症，预后良好。侵袭性和转移性EMPD患者并不常见，预后较差，即使对一线化疗有良好反应也是如此。已经尝试了多种化疗方案，效果各异，但尚未确立标准治疗方案。中枢神经系统似乎是常见的转移部位，其生存率高于内脏转移。我们报告一例转移性EMPD病例，该病例说明了治疗这种罕见疾病所面临的困难，目前尚无相关指南。
