Surgical Treatment and Outcomes of Patients With Extramammary Paget Disease: A Cohort Study

BACKGROUND Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma occurring mainly in the anogenital region. Traditional management with wide local excision has shown high recurrence rates, thus Mohs micrographic surgery (MMS) has emerged as a promising treatment option. OBJECTIVE To compare long-term outcomes after treatment with MMS or excision for primary EMPD. METHODS AND MATERIALS Retrospective cohort review was conducted for 207 patients with EMPD treated at Mayo Clinic in Rochester, MN, between 1961 and 2012. RESULTS Of the 25 patients treated with MMS, 19 primary tumors were included for outcome analysis, with an estimated 5-year recurrence-free survival rate of 91% (95% confidence interval [CI], 75-100) using Kaplan-Meier curve analysis. Of 158 patients treated with local excision, 124 were included for the analysis, with an estimated 5-year recurrence-free survival rate of 66% (95% CI, 56-78). The hazard ratio (HR) for association of treatment was 0.4 (95% CI, 0.10-1.65; p=.20). Estimated 5-year overall survival rates were 79% for MMS (95% CI, 61-100) and 68% for excision (95% CI, 59-78) (HR, 1.39 [95% CI, 0.69-2.82]; p=.36). CONCLUSION Although treatment of primary EMPD with MMS versus excision did not show statistical difference, MMS demonstrated favorable long-term outcomes and was associated with a higher recurrence-free survival rate.