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Breast-malignant, males, children
Juvenile papillomatosis (swiss cheese disease)
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Reviewer: Daniel Visscher, M.D., University of Michigan Hospitals, February 2009 (see Reviewers page)
Revised: 7 October 2009
Last major update: October 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Grossly distinct multinodular mass with clustering of cystic formations resembling swiss cheese, composed of epithelial proliferation and clustered cysts
● First described by Rosen in 1980 (AJSP 1980;4:3)
Terminology
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● Not part of WHO breast classification
Epidemiology
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● Mean age 19 years; 2/3 are less than age 20 years
Clinical
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● Resembles fibroadenoma clinically
● 10% develop breast carcinoma, higher risk if recurrent and bilateral disease and family history of breast cancer (AJCP 1990;93:599)
● 26-58% have positive family history of breast cancer (Cancer 1982;49:2591, AJCP 1986;86:745), 15% have bilateral disease
● May be associated with secretory carcinoma (Jpn J Clin Oncol 1985;15:457)
Case reports
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● Infant boy with Noonan syndrome and family history of breast cancer (Pediatr Blood Cancer 2005;45:991)
● 6 year old girl with coexisting secretory carcinoma (J Pediatr Surg 1987;22:637)
● 16 year old girl with ER-, PR+, HER2+ tumor and recurrences (Breast Cancer 1998;5:187)
Gross description
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● Localized multinodular mass with clustering of cystic formations resembling swiss cheese
Gross images
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Multiple cysts resembling swiss cheese
Microscopic description
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● Florid epithelial hyperplasia and papillomatosis, cysts with foamy histiocytes, sclerosing adenosis
● Variable apocrine metaplasia, atypia and necrosis
Micro images
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Clustered cysts
Epithelial hyperplasia
AFIP images
Multiple cystically dilated ducts Hyperplastic epithelium in dilated duct
Intraductal epithelial proliferation Apocrine metaplasia of hyperplastic
with necrosis intraductal epithelium
Hyperplastic epithelium and With sclerosing adenosis and apocrine
foam cells metaplasia adjacent to cystically dilated ducts
Multiple small intraductal papillomas #2 - high power shows hyperplastic intraductal
in 15 year old girl #1 epithelium, myoepithelial cells are associated with
the delicate fibrovascular stalks
Cytology description
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● Difficult to diagnose
● Cystic fluid, but mass persists after aspiration
● Sheets of hyperplastic breast epithelium with areas resembling fibroadenoma, macrophages and apocrine cells (Diagn Cytopathol 1993;9:457)
Additional references
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End of Breast – Malignant, Males, Children > Juvenile papillomatosis (swiss cheese disease)
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