Table of ContentsDefinition / 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 answer #1
Cite this page: Sanders MAG Paget disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed August 14th, 2020.
Definition / general
- In situ carcinoma involving the nipple epidermis
- 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
- "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)
- Present in 1 - 4% of all patients with breast carcinoma
- Affects men and women; age range is 27 to 88 (mean 54 - 63 years)
- 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
- 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
Contributed by Emily Reisenbichler, M.D.
Contributed by Mary Ann Gimenez Sanders, M.D, Ph.D.
Contributed by Dr. Semir Vranic - Paget with underlying DCIS
- 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)
- 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
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.