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Table of Contents
Definition / general | Terminology | Epidemiology | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Videos | Positive stains | Negative stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Paget's disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed July 14th, 2017.
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
- 53 year old woman with bilateral Pagetís disease derived from LCIS (Arch Pathol Lab Med 2002;126:90)
- 58 year old woman with vulvar and breast disease and invasive disease at both sites (Arch Gynecol Obstet 2009;280:313)
- 64 year old woman with nipple discharge and inverted nipple (Univ Pittsburgh Case #331)
- 83 year old woman with acinar pattern (Breast J 2007;13:520)
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
- Skin is fissured, oozing, ulcerated
- Resembles eczema (Breast Cancer Res Treat 2008;111:313)
Microscopic (histologic) 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
Microscopic (histologic) images
Scroll to see all images:
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
- AE1/3, CK7 (also stains Toker and Merkel cells, Am J Surg Pathol 1999;23:212)
- Low molecular weight keratin (CAM 5.2, AE1, Am J Surg Pathol 1992;16:58)
- EMA, CEA / MUC1 (Am J Surg Pathol 2001;25:1469, Am J Surg Pathol 2002;26:617)
- HER2 and androgen receptor (Mod Pathol 2005;18:1283)
- GCDFP-15 (50%)
- Variable p53, ER and PR
Electron microscopy description
- Intracytoplasmic lumina with microvilli, evidence of glandular differentiation
Differential diagnosis
- Benign proliferative nipple duct lesions or Toker cells: mucin- (APMIS 2008;116:139, Breast J 2009;15:394)
- Carcinoma in situ of skin / Bowenís disease: has individual cell keratinization and multinucleated giant cells, CK7-, mucin-, HER2-
- Clear cell keratinocytes: image
- Pemphigus vulgaris: clinically different (Breast J 2003;9:319)
- Superficial spreading melanoma: tumor cells invade dermis, S100+, HMB45+, AE1/3- (Dermatology Online Journal 13(2):18)
Additional references
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