Paget disease

Author: Elliot Weisenberg, M.D. (see Authors page)

Revised: 23 January 2018, last major update December 2013

Copyright: (c) 2003-2018,, Inc.

PubMed Search: Paget disease esophagus

Cite this page: Weisenberg, E. Paget disease. website. Accessed November 17th, 2018.
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