Breast malignant, males, children
In situ carcinoma
Paget's disease

Author: Dina Kandil, M.D. (see Authors page)

Revised: 18 October 2016, last major update August 2012

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Paget's disease [title] breast

Cite this page: Paget's disease. website. Accessed July 25th, 2017.
Definition / general
  • Malignant epithelial cells randomly disposed within epidermis of nipple / areola
  • A type of in situ carcinoma that arises in or involves the main excretory (lactiferous) ducts
  • Associated with underlying DCIS or invasive disease in almost all cases (Breast Cancer Res Treat 2008;112:513, Ann Surg Oncol 2005;12:391)
  • Described by Sir James Paget in 1874 as a crusted lesion of nipple caused by breast carcinoma
  • May derive from Toker cells (clear cells in nipple), or from epidermotropism of existing carcinoma (Hum Pathol 2003;34:1321)
  • Present in 1 - 4% of all patients with breast carcinoma; age ranges from 26 to 88, median age 54 years
  • Differs from Paget's disease of vulva (usually not associated with underlying carcinoma) and Paget's disease of bone
  • "Anaplastic Paget's", based on markedly atypical cells, is occasionally described (Am J Surg Pathol 1992;16:1085, J Cutan Pathol 2009;36:374), but not widely accepted as a distinct entity
  • Present in 1 - 2% of all patients with breast carcinoma, median age 54 years
Case reports
  • Mastectomy or possibly breast conserving surgery
  • Prognosis depends on underlying DCIS or invasive carcinoma
Clinical images

Images hosted on PathOut server:

Breast skin, courtesy of Mark R. Wick, M.D.

Images hosted on other servers:

Eczematoid lesion

Gross description
Microscopic (histologic) description
  • Single cells, groups or rarely tubules
  • Cells are large, atypical and spread throughout epidermis
  • Cells have abundant clear or light staining cytoplasm, abundant mucin and occasionally intracytoplasmic melanin (Melanoma Res 2004;14:S13, Am J Dermatopathol 2009;31:223)
  • Nucleus is large and vesicular with prominent nucleolus
  • DCIS is usually present and may also have invasive component
  • Underlying breast carcinoma is usually adjacent to Pagetís disease if one takes enough sections
Microscopic (histologic) images

Scroll to see all images:

Images hosted on PathOut server:
Courtesy of Mark R. Wick, M.D.:

Breast skin





AFIP images:

Large atypical cells in epidermis have round nuclei and prominent nucleoli

Involvement of terminal portion of lactiferous duct


Involvement of entire thickness
of epidermis, with pseudoepitheliomatous
hyperplasia and hyperkeratosis with involved
area, but not in normal appearing area

Images hosted on Flickr:
Contributed by Dr. Semir Vranic - Paget's with underlying DCIS:





Underlying DCIS (left to right): H&E, ER alpha, CK8, HER2


Images hosted on other servers:

Large atypical cells in epidermis

With hyperkeratosis and dermal inflammation

With underlying comedo DCIS

H&E and stains

Derived from LCIS

CK7+, E-cadherin negative



Positive stains
Negative stains
Electron microscopy description
  • Intracytoplasmic lumina with microvilli, evidence of glandular differentiation
Differential diagnosis
Additional references