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Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

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CURRENT ONCOLOGY REPORTS
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SPRINGER
DOI: 10.1007/s11912-023-01434-0

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Extramammary Paget disease; Imiquimod; Radiotherapy; Chemotherapy; HER-2

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This article reviews the purpose of the study, recent findings, and treatment approaches for Extramammary Paget disease (EMPD). EMPD is a rare tumor of uncertain origin, and treatment can involve various methods including surgery, radiotherapy, photodynamic therapy, chemotherapy, and targeted therapy. The article highlights the need for further research and diagnostic tools to guide therapeutic decisions for EMPD.
Purpose of ReviewExtramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1-2% of vulvar neoplasms. It is a primary cutaneous adenocarcinoma whose cell of origin is still a matter of controversy: it can either arise from apocrine/eccrine glands or from stem cells. The diagnosis demands a biopsy and entails a histopathological analysis by which cells show similar characteristics as breast Paget disease.Recent FindingsTreatment approach can entail surgery, radiotherapy, photodynamic therapy, systemic chemotherapy, and topical chemotherapy. For metastatic disease, many different chemotherapy regimens have been explored and even targeted therapy can play an important role in this disease. Since almost 30-40% of patients overexpress HER-2, trastuzumab and anti-HER-2 therapies can be employed in this setting.Due to its low incidence, there is almost no specific evidence on therapeutic interventions for this disease. Thus, there is a neat unmet need for molecular characterization of EMPD and diagnostic tools that allow clinicians to guide treatment both in the early and in the advanced disease settings. In this review, we aim to summarize available evidence about diagnosis and treatment of EMPD, both localized and metastatic, and to provide a comprehensive analysis that may help clinicians for therapeutic decisions.

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