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Breast-nonmalignant

Fibrocystic disease

Collagenous spherulosis of breast

 

Reviewer: Hind Nassar, M.D. in January 2009 (see Authors page)

Revised: 13 March 2014, last major update May 2010

Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.

 

Definition

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● Rare benign change occurring in proliferative lesions (papilloma, florid ductal hyperplasia) and characterized by intraluminal spherules (Am J Surg Pathol 1987;11:411)

 

Terminology

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● Also described as “adenoid cystic hyperplasia”

 

Epidemiology

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● In 1-2% of biopsies with hyperplastic lesions (Rosen’s Breast Pathology, page 130)

 

Etiology

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● The spherules consist of basement membrane material

 

Clinical features

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● Almost always an incidental finding, only rarely presents as a mass

● Associated with intraductal papilloma, sclerosing adenosis, radial sclerosing lesions; also LCIS (Am J Surg Pathol 1995;19:1366)

 

Case reports

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● 48 year old woman with adenomyoepithelioma (J Clin Pathol 2004;57:83)

● 54 year old woman with DCIS (Pathologica 2002;94:317)

 

Treatment

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● Requires no treatment by itself, but may be associated with malignancy

 

Gross description (Macroscopy)

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● Usually multifocal, 1 to 3 mm (not visible grossly)

 

Micro description (Histopathology)

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● Intraluminal clusters of eosinophilic or rarely basophilic (“mucinous spherulosis”, Arch Pathol Lab Med 1999;123:626), collagen-rich spherules within spaces, surrounded by flattened myoepithelial cells; spherules can calcify

● Associated with LCIS (25%) and microcalcifications (25%, Am J Surg Pathol 2006;30:20)

 

Micro images

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Tubular adenosis with features Within adenomyoepithelioma

of collagenous spherulosis

 

Adenoid cystic carcinoma (first column) vs. collagenous spherulosis (second column)

 

p63 c-kit p63 and c-kit

 

Other images: #1; #2; #3

 

Cytology description

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● Hyaline pink globules surrounded by benign myoepithelial cells in Giemsa stained smears (Cytopathology 2002;13:116)

Note: cytological evidence of spherulosis also suggests intraductal papilloma (Cytopathology 2009 Mar 7 [Epub ahead of print])

 

Mucinous spherulosis:

● Cribriform structures with lightly basophilic material embedded in loosely mucinous acellular background

● Intermediate to large, round hyaline spherules are naked or surrounded by myoepithelial cells

● Diff-Quik stain shows fibrillary radial appearance (Diagn Cytopathol 2006;34:626)

 

Positive stains

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Myoepithelial cells:

● Smooth muscle actin, smooth muscle myosin heavy chain, calponin

● p63 (nuclear staining, examine at high power, Mod Path 2006;19:1351)

 

Negative stains

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Myoepithelial cells:

● c-kit/CD117

 

Electron microscopy descriptions

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● Epithelial cells and myoepithelial cells plus associated basement membrane-like material (Breast J 2000;6:199)


Differential Diagnosis

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Adenoid cystic carcinoma: denser and tightly nodular material is rarely degenerated, stromal invasion, CD117+ luminal cells, calponin and smooth muscle myosin heavy chain negative, may have collagenous spherulosis-like structures (Pathol Int 2004;54:332)

Cribriform DCIS: multiple secondary lumens with round, regular spaces having sharp borders that appear to be made from “cookie-cutters”; no amorphous pink material within lumens

Signet ring variant of lobular carcinoma

 

Additional references

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Stanford University

 

End of Breast-nonmalignant > Fibrocystic disease > Collagenous spherulosis of breast

 

 

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