Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Paget’s Disease of the Anus

  • Denis ChatelainEmail author
  • Jean-François Fléjou
Reference work entry


Paget’s disease is a rare intraepithelial adenocarcinoma that may occur in the perianal skin. Its histogenesis is not completely understood. Paget’s disease could represent the epidermotropic spread of tumor cells from cancers of regional organs and from adenocarcinoma of skin adnexa such as eccrine or apocrine glands. It could also originate from an intraepidermal ectopic sweat gland or Bartholin’s glands’ epithelial cells or could originate from an in situ transformation of a pluripotential stem cell within the epidermis.

Perianal Paget’s disease was first reported in 1893 by Darier and Coulillaud. It encompasses two entities: primary and secondary Paget’s disease.

Secondary perianal Paget’s disease represents half of the cases. It results from the epidermotropic spread of an underlying neoplasm such as adnexal adenocarcinoma or an internal malignancy mainly gastrointestinal carcinoma (usually anal or rectal cancer) or genitourinary carcinoma (mainly urethral carcinoma)....

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References and Further Reading

  1. Goldblum, J. R., & Hart, W. R. (1998). Perianal Paget’s disease: A histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma. The American Journal of Surgical Pathology, 22, 170–179.CrossRefGoogle Scholar
  2. Shia, J. (2010). An update on tumors of the anal canal. Archives of Pathology & Laboratory Medicine, 134, 1601–1611.Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Service d’Anatomie PathologiqueCentre Hospitalier et Universitaire du NordAmiensFrance
  2. 2.Faculté de Médecine Pierre et Marie Curie, Service d’Anatomie et Cytologie PathologiquesHôpital Saint-AntoineParisFrance