Extramammary Paget Disease of External Genitalia: Surgical Excision and Follow-up Experiences With 19 Patients

PURPOSE There are only a few reports of extramammary Paget disease (EMPD) of the external genitalia because it is a rare malignancy. We investigated patients with EMPD of the penis and scrotum and report the outcome of surgical management. MATERIALS AND METHODS From 2000 to 2012, a total of 19 patients diagnosed as having penile and scrotal EMPD underwent wide local excision with or without intraoperative frozen biopsy or preoperative mapping biopsy. The medical charts of these patients were reviewed and analyzed retrospectively. Mean follow-up was 22.5 months (range, 1 to 60 months). RESULTS The mean age of the patients was 68 years (range, 57 to 82 years). In some patients, the lesions were misdiagnosed as either eczema or some other benign skin lesion at outside institutions, with a mean delay in diagnosis of 43.5 months (range, 1 to 198 months). Intraoperative frozen biopsy or preoperative mapping biopsy was performed in 18 patients. The resection margin was negative in 9 patients (47.4%) and positive in 10 patients (57.6%). Intraepithelial EMPD without dermis invasion was observed in 5 patients (26.3%), whereas diseases with dermis invasion were noted in 14 patients (73.7%). During the follow-up period, recurrences occurred in four patients, and two patients with dermis invasion and recurrence died from the disease. CONCLUSIONS Diagnosis of EMPD should not be delayed to allow for prompt management. Our findings suggest that intraoperative frozen biopsy or preoperative mapping biopsy cannot guarantee negative margins on final pathology. However, preoperative mapping biopsy and wide local excision with intraoperative frozen biopsy demonstrates good prognosis of EMPD, especially in those cases without dermal invasion.