Vulvar Paget's disease: prognostic factors and survival outcomes from a retrospective cohort analysis

Vulvar Paget's disease (VPD) is a rare intra-epithelial neoplasm with a high recurrence rate and uncertain prognostic factors. This retrospective study aimed to evaluate disease-free survival (DFS) and overall survival (OS) in 27 patients diagnosed with VPD, assessing the impact of surgical type, disease extent, resection margins, lymphadenectomy, wound dehiscence and age. Kaplan-Meier analysis and Cox proportional hazards regression were used to determine survival outcomes and prognostic factors. The results revealed that surgical type had a significant influence on OS, with patients undergoing simple excision/vulvectomy achieving better survival outcomes than those who underwent radical vulvectomy (p = 0.036). Age was also identified as a significant predictor of OS, with increasing age associated with reduced risk of mortality (p = 0.017). However, no significant associations were found between DFS and any of the clinical factors examined, except for borderline significance for wound dehiscence (p = 0.061). The literature review confirmed the high recurrence rate and heterogeneity in outcomes. These findings reinforce the need for individualized treatment, as radical surgery did not confer a survival advantage. Given the emerging role of molecular biomarkers such as HER2 and PD-L1 in VPD, future studies should explore targeted therapies to optimize treatment strategies. Larger, multi-centric studies with extended follow-up are needed to refine prognostic factors and improve patient outcomes.