Paget disease

Topic Completed: 1 December 2013

Minor changes: 27 February 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Paget disease esophagus

Elliot Weisenberg, M.D.
Page views in 2019: 386
Page views in 2020 to date: 312
Cite this page: Weisenberg E. Paget disease. website. Accessed July 13th, 2020.
Definition / general
  • Carcinoma characterized by the presence of malignant glandular epithelial cells within the squamous epithelium of the esophagus (paralleling WHO definition of Paget disease of nipple)
  • While Paget disease in the nipple has been reported without underlying malignancy, essentially all reported cases in the esophagus have underlying carcinoma
  • May occur in 5% of esophageal resections for adenocarcinoma
  • Cases reported with underlying adenocarcinoma, mucous gland carcinoma and adenosquamous carcinoma (Am J Surg Pathol 2008;32:1068)
Clinical features
  • Occurs due to spread from underlying malignancy
  • In rare cases, may serve as a marker of underlying malignancy
  • Endoscopic biopsy or resection
Prognostic factors
  • Prognosis related to underlying malignancy
Case reports
Microscopic (histologic) description
  • Glandular differentiation or mucin production by intraepithelial Paget cells
Positive stains
Negative stains
Differential diagnosis
Back to top