Breast malignant, males, children
In situ carcinoma
Paget disease

Editorial Board Member: Emily S. Reisenbichler, M.D.
Mary Ann Gimenez Sanders, M.D, Ph.D.

Topic Completed: 1 July 2017

Revised: 6 May 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Paget disease [title] breast

Mary Ann Gimenez Sanders, M.D, Ph.D.
Page views in 2018: 22,637
Page views in 2019 to date: 20,191
Cite this page: Sanders MAG Paget disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed September 18th, 2019.
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

Contributed by Mark R. Wick, M.D.

Breast skin



Images hosted on other servers:

Eczematoid lesion

Gross description
  • Nipple skin with scaling crust, erythema and exudate resembling chronic eczema
  • May have ulceration
Gross images

Contributed by Dr. Emily Reisenbichler

Paget disease of the nipple

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

Contributed by Emily Reisenbichler, M.D.

Paget disease of the nipple, low and high power



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



Contributed by Dr. Semir Vranic - Paget with underlying DCIS

H&E

CK8+

HER2+

S100-

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.
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