Clinicopathologic Features and Treatment Outcomes of Primary Extramammary Paget Disease of the Vulva

OBJECTIVE: The aim of this study was to identify the clinicopathologic features and treatment outcome of primary extramammary Paget disease of the vulva (EMPDV). MATERIALS AND METHODS: We performed a retrospective analysis of 14 patients who were treated at our center from April 1994 to November 2010. RESULTS: The mean age of patients in our sample was 64.4 years (range = 47-83 y). We observed intraepithelial and invasive EMPDV in 11 (78.6%) and 3 (21.4%) patients, respectively. Moreover, we observed a positive incision margin in 9 patients (64.3%). During a median follow-up period of 69.5 months (range = 32-221 mo), we observed recurrence in 3 patients (21.4%), 2 of whom had invasive EMPDV and 1 had intraepithelial EMPDV. Time to recurrence was 16 and 18 months for patients with invasive EMPDV and 98 months for patients with intraepithelial EMPDV. The recurrence rate of intraepithelial EMPDV and invasive EMPDV was significantly different (9.1% and 66.7%, respectively, p < .028). Local recurrence occurred in all 3 patients, necessitating further surgical resection. One patient with recurrence of invasive EMPDV received adjuvant radiotherapy but died 101 months after the initial treatment. The other 2 patients remained alive without recurrence. We did not observe distant recurrence. CONCLUSIONS: The recurrence rate of invasive EMPDV was high. However, because distant metastasis is rare, repeat surgical excision for recurrent EMPDV and long-term observation are necessary for a good prognosis.