<h3>Objectives:</h3> To examine the association between surgical margin status and recurrence pattern in invasive vulvar Paget's disease. <h3>Methods:</h3> This is a preplanned secondary analysis of previously organized nationwide retrospective observational study in Japan (JGOG-1075S). Women with stage I-IV invasive vulvar Paget's disease who received surgical treatment from 2001-2010 were examined (n=139). Multivariable analysis was used to assess local-recurrence, distant-recurrence, and all-cause mortality based on surgical margin status. <h3>Results:</h3> The median age was 70 years. The majority had stage I disease (61.2%), and the median tumor size was 5.0cm. Nodal metastasis was observed in 15.1%. Simple vulvectomy (46.0%) was the most common surgery type followed by radical vulvectomy (28.1%). More than half received vulvar reconstructive surgery (59.0%). Positive surgical margin was observed in 35.3%, and close margin <1cm was observed in 29.5%. Vulvectomy type was not associated with surgical margin status (<i>P</i>=0.424). The median follow-up was 5.8 years, and positive surgical margin was significantly associated with local-recurrent (5-year cumulative rates for positive versus negative margin: 38.0% versus 15.0%, <i>P</i>=0.005) but not distant-recurrence (20.5% versus 16.0%, <i>P</i>=0.388). Positive surgical margin was also associated with all-cause mortality (5-year overall survival rates for positive versus negative margin: 72.6% versus 88.2%, <i>P</i>=0.032). In multivariable analysis, positive surgical margin remained an independent factor associated with increased risk of local-recurrence (hazard ratio 3.32, 95% confidence interval 1.29-8.53) and all-cause mortality (hazard ratio 2.87, 95% confidence interval 1.20-6.83). <h3>Conclusions:</h3> Positive surgical margin appears to be common in invasive vulvar Paget's disease that is associated with increased local-recurrence and all-cause mortality risks. Role of alternative surgical technique (e.g., Mohs micrographic surgery) or adjuvant therapy (e.g., imiquimod) merits further investigation to improve local disease control.
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