Breast malignant, males, children
In situ carcinoma
Paget disease

Author: Mary Ann Gimenez Sanders, M.D., Ph.D. (see Authors page)
Editorial Board Member Review: Emily Reisenbichler M.D. (see Authors page)

Revised: 17 August 2017, last major update July 2017

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Paget disease [title] breast

Cite this page: Sanders, M. A. G. Paget disease. PathologyOutlines.com website. http://pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed September 23rd, 2017.
Definition / general
  • In situ carcinoma involving the nipple epidermis
Essential features
  • Clinically presents as a nipple rash
  • Usually diagnosed on skin punch or shave biopsy of the nipple
  • Paget cells originate from DCIS in lactiferous ducts
  • Majority of cases are associated with DCIS or invasive carcinoma deeper in the breast
Terminology
  • "Secondary" Paget disease occurs when invasive carcinoma arising deep in the breast directly invades the skin; the invasive carcinoma is usually large in size
  • Paget disease with dermal invasion occurs when Paget cells cross the basement membrane of the epidermis; the invasive component is usually small in size (Arch Pathol Lab Med 2013;137:72)
Epidemiology
  • Present in 1 - 4% of all patients with breast carcinoma
  • Affects men and women; age range is 27 to 88 (mean 54 - 63 years)
Case reports
Treatment
  • Nipple areolar complex is removed by either mastectomy or central lumpectomy with or without sentinel lymph node biopsy
  • Treatment, including surgical and adjuvant therapy, as well as prognosis, depend on underlying DCIS or invasive carcinoma
Clinical images

Images hosted on PathOut server:

Breast skin, contributed by Mark R. Wick, M.D.



Images hosted on other servers:

Eczematoid lesion

Gross description
  • Nipple skin with scaling crust, erythema and exudate resembling chronic eczema
  • May have ulceration
Microscopic (histologic) description
  • Single cells or clusters of cells spread throughout the epidermis
  • Cells have abundant pale cytoplasm, irregular large nucleus with prominent nucleoli
  • Underlying dermis with chronic inflammation
  • Epidermis with hyperkeratosis and possibly ulceration
  • Florid cases can show gland formation
Microscopic (histologic) images

Scroll to see all images:


Images contributed by Mary Ann Gimenez Sanders, M.D, Ph.D.

Secondary Paget Disease

Lactiferous duct of the nipple with DCIS

Epidermal hyperkeratosis

Epidermal ulcer


Florid Paget disease

Paget cells with pale cytoplasm

Paget disease with dermal invasion

HER2 positive Paget Disease


Images hosted on PathOut server:

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



Contributed by Dr. Semir Vranic - Paget with underlying DCIS:

H&E

CK8+

HER2+

S100-


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

CK7+



Contributed by Mark R. Wick, M.D.:

Breast skin


Pseudobullous

Invasive

PAS

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+

Positive stains
Negative stains
Videos



Differential diagnosis
Sample report
  • Diagnosis: Ductal carcinoma in situ involving the nipple epidermis as Paget disease
Board review question #1
Which immunohistochemical stain is most likely to be positive in Paget disease?

  1. CK5 / 6
  2. CK20
  3. ER
  4. HER2
  5. PR
Board review answer #1
D. HER2: the majority of Paget disease cases are HER2 positive and therefore HER2 can be used to confirm the diagnosis. In the absence of invasive carcinoma, HER2 positivity does not influence treatment since Paget disease is an in situ carcinoma. Paget disease is negative for CK5 / 6 and CK20. ER and PR show variable expression.