Track

Abstract

Introduction: Extramammary Paget disease (EMPD) is a rare cutaneous carcinoma usually presenting as a genital erythematous lesion in the elderly. Methods: Clinical and pathological features of 93 patients with primary EMPD from 1990 to 2025 were retrieved from the medical records to determine survival prognostic factors. Results: 71 (76.3%) patients are female and 22 (23.7%) are male. The ages range from 35 to 98 years (mean, 72.2; median, 73). The sites of the tumor include vulva (91.6% of females), penoscrotal region (40.9% of males), perineum (7.5%), anal/perianal area (11.8%), inguinal region (14.0%), lower extremities (4.3%), axilla (3.2%), and neck (1.0%). Cutaneous presentations include erythema (59.6%), eczematous lesion (13.5%), erosion/ulceration (36.0%), pruritus (34.8%), hypopigmentation (12.4%), and others (27.0%). Follow-up intervals range from 0 to 324 months (median, 74 [IQR 21-132]).  In univariate Cox proportional hazard models, variables significantly correlated with worse progression-free survival (PFS) include size ≥ 4cm (HR 1.10 [95%CI 1.01-1.19]; p=0.032), vulva involvement (HR 2.36 [95%CI 1.03-5.39]; p=0.042), bilateral lesion (HR 3.97 [95%CI 2.02-7.78]; p<0.001), pruritus (HR 2.27 [1.15-4.49]; p=0.018), positive margin (HR 3.33 [95%CI 1.18-9.44]; p=0.023), and radical surgery (HR 3.30 [95%CI 1.68-6.47]; p<0.001). However, only bilateral lesions remained significant in multivariate analyses (HR 3.0 [95%CI 1.29-6.99]; p=0.011). Only age ≥ 73 years correlated significantly with worse overall survival (OS) in univariate analyses (HR 2.91 [95%CI 1.13-7.48]; p=0.027). Conclusion:  For primary EMPD, bilateral disease is a significant predictor of worse disease progression, whereas age of 73 years or more is a significant predictor of worse overall survival.