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, PathologyOutlines.com, Inc.

PubMed Search: Paget's disease [title] breast

Cite this page: Paget's disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed December 6th, 2016.
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
Terminology
  • 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
Epidemiology
  • Present in 1 - 2% of all patients with breast carcinoma, median age 54 years
Case Reports
Treatment
  • 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
Micro 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
Micro Images

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

Breast skin



Pseudobullous

Invasive

PAS

CK7



AFIP images:

Large atypical cells in epidermis have round nuclei and prominent nucleoli

Involvement of terminal portion of lactiferous duct

HER2+

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:

H&E

CK8+

HER2+

S100-



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

CK7+



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

CK7+

Videos



Positive Stains
Negative Stains
Electron Microscopy Description
  • Intracytoplasmic lumina with microvilli, evidence of glandular differentiation
Differential Diagnosis
Additional References