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Breast-malignant, males, children
Morphologic variants of DCIS
Paget’s disease
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Reviewer: Daniel Visscher, M.D., University of Michigan Hospitals, February 2009 (see Reviewers page)
Revised: 30 August 2009
Last major update: August 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
See also Anaplastic Paget’s Disease
Definition
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● 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 Path 2003;34:1321)
Terminology
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● Differs from Paget’s disease of vulva (usually not associated with underlying carcinoma) and Paget’s disease of bone
Epidemiology
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● Present in 1-2% of all patients with breast carcinoma, median age 54 years
Treatment and prognosis
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● Mastectomy or possibly breast conserving surgery
● Prognosis depends on underlying DCIS or invasive carcinoma
Case reports
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● 53 year old woman with bilateral Paget’s disease derived from LCIS (Archives 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)
Clinical images
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Eczematoid lesion Pigmented lesion Eczema-like lesion
Gross description
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● Skin is fissured, oozing, ulcerated
● Resembles eczema (Breast Cancer Res Treat 2008;111:313)
Microscopic description
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● 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 Paget’s disease if one takes enough sections
Micro images
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Large atypical cells in epidermis
With hyperkeratosis and dermal inflammation With underlying comedo DCIS
H&E and stains With melanin pigment Derived from LCIS
CK7+, E-cadherin negative CK7+
EMA+ HER2+
AFIP images:
Involvement of entire thickness of epidermis, Large atypical cells in epidermis have
with pseudoepitheliomatous hyperplasia and round nuclei and prominent nucleoli
hyperkeratosis with involved area, but not in
normal appearing area
Involvement of terminal HER2+
portion of lactiferous duct
Virtual slides
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Paget’s disease
Videos
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Positive stains
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● CK7 (also stains Toker and Merkel cells, AJSP 1999;23:212),
● Low molecular weight keratin (CAM 5.2, AE1, AJSP 1992;16:58)
● EMA, CEA / MUC1 (AJSP 2001;25:1469, AJSP 2002;26:617)
● HER2 and androgen receptor (Mod Path 2005;18:1283)
● GCDFP-15 (50%)
● Variable p53 and ER
Negative stains
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● MUC2, MUC5AC (mucin expressed in extramammary Paget’s disease), S100, HMB45
Electron microscopy
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● Intracytoplasmic lumina with microvilli, evidence of glandular differentiation
Differential diagnosis
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● Carcinoma in situ of skin / Bowen’s disease - has individual cell keratinization and multinucleated giant cells, CK7-, mucin-
● Superficial spreading melanoma - tumor cells invade dermis (Dermatology Online Journal 13(2):18)
● Benign proliferative nipple duct lesions or Toker cells (APMIS 2008;116:139, Breast J 2009;15:394)
● Pemphigus vulgaris - clinically different (Breast J 2003;9:319)
● Clear cell keratinocytes (image)
Additional references
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End of Breast – Malignant, Males, Children > Paget’s disease
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