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

Abstract

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.