Ann Dermatol.  2016 Oct;28(5):624-628. 10.5021/ad.2016.28.5.624.

Perianal and Vulvar Extramammary Paget Disease: A Report of Six Cases and Mapping Biopsy of the Anal Canal

• Nagai Y
• Kazama S
• Yamada D
• Miyagawa T
• Murono K
• Yasuda K
• Nishikawa
• Tanaka T
• Kiyomatsu
• Hata K
• Kawai K
• Masui Y
• Nozawa H
• Yamaguchi
• Ishihara S
• Kadono T
• Watanabe T

• KMID: 2382888
• DOI: http://doi.org/10.5021/ad.2016.28.5.624

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.

Keyword

Anal canal; Biopsy; Extramammary Paget disease

MeSH Terms

Anal Canal*
Biopsy*
Colonoscopy
Consensus
Follow-Up Studies
Humans
Paget Disease, Extramammary*
Rectum
Recurrence
Skin

Figure

• 

  Fig. 1 Mapping biopsy of the anal canal. (A) A 69-year-old female with perianal extramammary Paget disease (EMPD) (the second patient). (B) The anal canal was sufficiently enlarged using a retractor in the jack-knife position under spinal anesthesia, and mapping biopsy of the anal canal was performed. (C) A scheme of the mapping biopsy of the anal canal. A total of 12 biopsies were performed (indicated by crosses and small circles): four on the dentate line, four 0.5 cm above the dentate line, and four 1.0 cm above the dentate line. The small circles demonstrate the sites that were positive for Paget cells. The large round circle indicates the anal verge, and the wavelike circle indicates the dentate line. (D) A 75-year-old female with vulvar EMPD (the sixth patient). The lesion involved the anal verge, and spread of Paget cells into the anal canal was highly suspected. (E) The anal canal was sufficiently enlarged using a retractor in the jack-knife position under spinal anesthesia. The spread of Paget cells into the anal canal were particularly suspected in the vertebral side. (F) A scheme of the mapping biopsy of the anal canal. A total of 16 biopsies were performed: five on the anal verge, three 10 mm above the anal verge, five on the dentate line, and three 10 mm above the dentate line. The small circles demonstrate the sites that were positive for Paget cells.

• 

  Fig. 2 Surgical findings of a 69-year-old female with perianal extramammary Paget disease (the second patient). (A) Wide local excision deep to the subcutaneous fat was performed. (B) Reconstruction was performed with split thickness skin grafts. (C) Surgically resected specimen.

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