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

Nomura, Hidetaka MD; Matoda, Maki MD; Okamoto, Sanshiro MD; Kondo, Eiji MD; Omatsu, Kohei MD; Kato, Kazuyoshi MD; Takeshima, Nobuhiro MD

Journal of Lower Genital Tract Disease: April 2015 - Volume 19 - Issue 2 - p 145–148
doi: 10.1097/LGT.0000000000000063
Original Articles: Vulva and Vagina

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.

Repeat surgical excision for recurrent extramammary Paget disease of the vulva and long-term observation are necessary.

Department of Gynecology, Cancer Institute Hospital, Tokyo, Japan

Reprint requests to: Hidetaka Nomura, MD, Department of Gynecology,Cancer Institute Hospital, Ariake 3-8-31, Koto-ku, Tokyo 135-8550, Japan. E-mail:

The authors have declared that they have no conflict of interest.

The authors did not receive financial support for this study.

Copyright © 2015 by the American Society for Colposcopy and Cervical Pathology