Breast
Ductal carcinoma in situ
Paget disease
Editorial Board Member: Emily S. Reisenbichler, M.D.
Topic Completed: 1 July 2017
Minor changes: 16 February 2021
Copyright: 2002-2019, PathologyOutlines.com, Inc.
PubMed Search: Paget disease [title] breast
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Videos | Differential diagnosis | Sample report | Board review style question #1 | Board review style answer #1Cite this page: Sanders MAG Paget disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed March 4th, 2021.
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
- 53 year old woman with bilateral Paget disease derived from LCIS (Arch Pathol Lab Med 2002;126:90)
- 91 year old man with mammary Paget disease (Surg Case Rep 2015;1:103)
- CK7 negative Paget disease, a potential diagnostic pitfall (Pathol Res Pract 2016;212:279)
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-
Videos
Differential diagnosis
- Carcinoma in situ of skin / Bowen disease: individual cell keratinization and multinucleation, CK5 / 6+, CK7-, CEA-, HER2-
- Melanoma in situ: MelanA+, HMB45+, CK7-
- Toker cells and pagetoid dyskeratosis (benign clear and pale cells of the nipple epidermis): bland appearing cells, HER2- (Histol Histopathol 2009;24:367)
Sample report
- Diagnosis: ductal carcinoma in situ involving the nipple epidermis as Paget disease
Board review style question #1
Which immunohistochemical stain is most likely to be positive in Paget disease?
- CK5 / 6
- CK20
- ER
- HER2
- PR
Board review style 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.
