Extramammary Paget's disease of penis and scrotum

Objectives. To make clear the uncertainty of the clinical outcome of extramammary Paget's disease (EMPD). Penile and scrotal involvement of EMPD is exceedingly rare, and only small series or case reports have been reported. Methods. From 1995 to 2003, 36 patients with penile and scrotal EMPD were treated and followed up. Local wide excision was done in all patients with or without intraoperative frozen biopsy analysis. Results. Of the 36 patients, 13 (36.1%) underwent intraoperative frozen biopsy analysis and only 1 patient (7.7%) had a positive surgical margin. However, 23 (63.9%) underwent local wide excision with excessive surgical margins of up to 1 to 2 cm only by gross examination, but 17 (73.9%) of them had positive surgical margins (P <0.01). Of the 17 patients with positive surgical margins, 8 developed local recurrence at a median of 8 months of follow-up (P <0.05). One patient who had invasion to the subcutaneous tissue died of metastatic EMPD and internal malignancy (renal cell carcinoma) at 17 months after the initial operation. No patient had underlying adnexal carcinoma. Conclusions. The results of our study indicate that local wide excision with intraoperative frozen biopsy analysis is essential to the complete treatment of EMPD.