Journal of Gastrointestinal Surgery

, Volume 16, Issue 10, pp 1967–1971

Extramammary Paget Disease in Peristomal Skin: Report of a Unique Case

  • Xiuli Liu
  • Genevieve B. Melton
  • Hao Xie
  • David W. Dietz
Case Report



Extramammary Paget disease is a rare neoplasm most often found in the perianal area which can be primary in origin or secondary from underlying colorectal adenocarcinoma with epidermotropic spreading. However, the occurrence of extramammary Paget disease has not been described in peristomal skin.


We describe a single case of Paget disease in the peristomal skin of a 61-year-old woman with ulcerative colitis who underwent a proctocolectomy 33 years prior to now with chronic intestinal obstruction and an area of “ectopic mucosa” adjacent to her ileostomy. The patient was taken to surgery for exploratory laparotomy and revision of her stoma.


Macroscopic and microscopic examinations of the peristomal lesion demonstrated Paget disease with intraepidermal spreading of dysplastic intestinal/glandular cells but no invasive adenocarcinoma in the peristomal skin after extensive sampling.


To our knowledge, this is the first reported case of extramammary Paget disease occurring in peristomal skin. The current case emphasizes the importance of recognizing such disease and the potential for associated neoplasia in patients with intestinal stomas.


Extramammary paget disease Colorectal adenocarcinoma Peristomal skin CDX2 

Copyright information

© The Society for Surgery of the Alimentary Tract 2012

Authors and Affiliations

  • Xiuli Liu
    • 1
  • Genevieve B. Melton
    • 2
  • Hao Xie
    • 3
  • David W. Dietz
    • 4
  1. 1.Department of Anatomic PathologyCleveland ClinicClevelandUSA
  2. 2.Department of SurgeryUniversity of MinnesotaMinneapolisUSA
  3. 3.Cleveland Clinic Lerner College of Medicine of Case Western Reserve UniversityClevelandUSA
  4. 4.Department of Colorectal SurgeryCleveland ClinicClevelandUSA

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