Extramammary Paget's Disease of Penis and Scrotum: Diagnosis, Treatment and Prognosis

Yang, Won Jae; Kim, Dong Suk; Im, Young Jae; Cho, Kang Su; Rha, Koon Ho; Cho, Nam Hoon; Choi, Young Deuk

Abstract

PURPOSE: Penile and scrotal involvements in extramammary Paget's disease (EMPD) are exceedingly rare, with only small series or case reports having been reported in the literature. Our study was undertaken to clarify the uncertainty of the clinical outcome of this disease.
MATERIALS AND METHODS
Between 1995 and 2003, 32 patients with penile or scrotal EMPD were treated and followed up. Local wide excision was performed in all patients, with or without an intraoperative frozen biopsy.
RESULTS
Of the 32 patients, 12 (37.5%) underwent local wide excision with an intraoperative frozen biopsy; 1 patient (8.3%) had a positive surgical margin. Conversely, 20 (62.5%) underwent local wide excision with an excessive surgical margin of upto 1 to 2cm only due to the gross findings, but 15 (75.0%) had a positive surgical margin (p<0.001). 8 of the 16 patients with a positive surgical margin had a local recurrence at the 8 month follow-up (p<0.05). One patient with invasion to the subcutaneous tissue had died of metastatic EMPD and internal malignancy (renal cell carcinoma) at the 17 month postoperative follow-up. There was no underlying adnexal carcinoma in any case.
CONCLUSIONS
Local wide excision with intraoperative frozen biopsy was essential for the complete treatment of EMPD. There was no associated internal malignancy or underlying adnexal carcinoma at the initial diagnoses.