BACKGROUND: Extramammary Paget's disease (EMPD) is an uncommon tumor that usually occurs on the genitalia. It almost always extends beyond clinically apparent margins and has a high rate of recurrence. OBJECTIVE: To establish treatment guidelines for EMPD in Asian patients. METHODS: A retrospective review was done on pertinent demographic data, tumor data, treatment characteristics, and follow-up data of 35 patients between 1996 and 2006. Review of literature for treatment modalities and recurrence rates of EMPD was also performed. RESULTS: Thirty-four of the 35 patients (30 men and 5 women) had lesions in the genital area and one patient in the axilla. Mean follow-up duration was 62.7 months (8-156 months) and two of 11 (18.2%) recurred after Mohs micrographic surgery (MMS), compared with eight recurrences of 22 (36.4%) after standard wide excision. Two patients treated with nonsurgical modalities did not achieve complete remission. Estimated 5-year tumor-free rate using Kaplan-Meier graph was 69.7% in all patients, with a rate of 81.8% for MMS and 63.6% for wide excision. CONCLUSIONS: MMS is more effective, with lower recurrence rate than wide excision, and should be regarded as the first-line treatment for nonmetastatic EMPD.
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