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Breast malignant, males, children

Childhood tumors

Juvenile papillomatosis (swiss cheese disease)


Reviewer: Monika Roychowdhury, M.D. (see Reviewers page)
Revised: 18 November 2012, last major update August 2012
Copyright: (c) 2001-2012, 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 (Am J Surg Pathol 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 features
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● Resembles fibroadenoma clinically
● 10% develop breast carcinoma, higher risk if recurrent, bilateral disease and family history of breast cancer (Am J Clin Pathol 1990;93:599)
● 26-58% have positive family history of breast cancer (Cancer 1982;49:2591, Am J Clin Pathol 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)
● 17 year old boy (G Chir 2011;32:374)

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

Micro description
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● Florid epithelial hyperplasia and papillomatosis, cysts with foamy histiocytes and sclerosing adenosis
● Variable apocrine metaplasia, atypia and necrosis

Micro images
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Various images

   
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             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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Stanford University

End of Breast malignant, males, children > Childhood tumors > Juvenile papillomatosis (swiss cheese disease)


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