title: Perianal and Vulvar Extramammary Paget Disease: A Report of Six Cases and Mapping Biopsy of the Anal Canal.
abstract: Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Yuzo
authors.initials: Y
authors.lastname: Nagai
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Sinsuke
authors.initials: S
authors.lastname: Kazama
authors.affiliation: Department of Dermatology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Yamada
authors.affiliation: Department of Dermatology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Takuya
authors.initials: T
authors.lastname: Miyagawa
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Murono
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Yasuda
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nishikawa
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Tanaka
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Tomomichi
authors.initials: T
authors.lastname: Kiyomatsu
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Hata
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Kazushige
authors.initials: K
authors.lastname: Kawai
authors.affiliation: Department of Dermatology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Masui
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Nozawa
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Hironori
authors.initials: H
authors.lastname: Yamaguchi
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Soichiro
authors.initials: S
authors.lastname: Ishihara
authors.affiliation: Department of Dermatology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kadono
authors.affiliation: Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Watanabe
keywords: Anal canal; Biopsy; Extramammary Paget disease