Extramammary Paget Disease: A Single-Center, Clinical Analysis

BACKGROUND: Extramammary Paget disease (EMPD) is a rare cutaneous malignancy that typically presents on apocrine-rich skin, especially the genitals. Surgery is often the first-line treatment, but other nonsurgical options may be considered. Historically, local recurrence rates are high. OBJECTIVE: To describe and analyze the clinical characteristics and outcomes of EMPD based on anatomic site of presentation. METHODS: All cases of EMPD were identified from the Washington University in St. Louis institutional pathology database between January 2000 and December 2021. Medical records were manually reviewed to identify clinical data for analysis. RESULTS: Eighty patients were identified. The vulva was the most common site of presentation. Despite a majority of vulvar EMPD cases being treated with radical excision, almost 95% of patients had positive surgical margins, and vulvar cases experienced the highest rate of local recurrence. Metastatic progression was more common in perianal and penoscrotal EMPD. CONCLUSION: EMPD is a rare malignancy with an overall good prognosis that has potential to act aggressively in certain cases. Although recurrence rates are higher in vulvar EMPD, perianal and penoscrotal EMPD appear to behave more aggressively. Margin-controlled surgery may lead to better outcomes, and adjuvant therapies can be considered when clear margins are not feasible.