Abstract
BackgroundExtramammary Paget’s disease (EMPD) sometimes spreads from the skin to mucosal areas, and curative surgical excision of these areas is challenging. The aim of this study is to analyze the impact of mucosal involvement and surgical treatment on the survival of patients with EMPD.MethodsWe conducted a retrospective review of 217 patients with EMPD. We also assessed the associations between tumor involvement in boundary areas (anal canal, external urethral meatus, vaginal introitus), prognostic factors, and survival in 198 patients treated with curative surgery.ResultsOf 217 patients, 75 (34.6%) had mucosal boundary area involvement. Lesions in these areas were associated with frequent lymphovascular invasion (p = 0.042), lymph node metastasis (p = 0.0002), incomplete excision (p < 0.0001), and locoregional recurrence (p < 0.0001). Boundary area involvement was an independent prognostic factor associated with disease-specific survival, per multivariate analysis (HR: 11.87, p = 0.027). Incomplete excision was not significantly correlated with disease-specific survival (HR: 1.05, p = 0.96).ConclusionBoundary area tumor involvement was a major risk factor for incomplete excision, local recurrence, and poor survival outcomes. However, incomplete removal of primary tumors was not significantly associated with poor prognosis. A less invasive surgical approach for preserving anogenital and urinary functions may be acceptable as the first-line treatment for resectable EMPD.
Highlights
Extramammary Paget’s disease (EMPD) is a rare neoplastic condition [1]
Approximately 15–40% of EMPD lesions display dermal invasion, which is known as invasive EMPD, and this increases the risk of lymph node and distant metastasis [2, 4]
We showed that lesions involving boundary areas were associated with high risk for poor survival outcomes, regardless of whether complete surgical removal was achieved, and that incomplete excision of EMPD did not affect patient outcomes
Summary
Extramammary Paget’s disease (EMPD) is a rare neoplastic condition [1]. It commonly affects areas rich in apocrine sweat glands, including the vulva, perineal area, perianal area, scrotal area, and penile skin [1, 2]. Most EMPD tumors are restricted to the epidermis as in situ lesions, and they are associated with good prognosis because of their slow-growing nature [1, 8]. Extramammary Paget’s disease (EMPD) sometimes spreads from the skin to mucosal areas, and curative surgical excision of these areas is challenging.
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