Extramammary Paget's disease in Chinese males: a 21-year experience.

Background: Extramammary Paget's disease (EMPD) is a rare disease in Chinese males. Our objectives were to examine the results of our experience, to correlate them with the published literature, and thus to formulate improved management guidelines.

Methods: This was a retrospective analysis of all male patients with EMPD treated at our institution. We reviewed the patients and their case notes for data such as presenting symptoms, delay to diagnosis, incidence of concurrent malignancies, treatments given, and a variety of outcome measures, including the number of patients with recurrent or residual disease.

Results: The overall recurrence rate was 35%. The risk of recurrence was reduced if intraoperative frozen section analysis was used (25%) compared to simple wide local excision (60%). Wide local excision failed to excise the disease completely in 38%. There were no associated internal malignancies and no disease-related deaths in our sample.

Conclusions: We recommend intraoperative frozen sections to reduce the risk of incomplete resection and to reduce the rate of recurrence. In our population, screening for internal malignancies can be restricted to select patients, e.g., those with perianal disease, those with invasive disease, and young patients.