Breast malignant, males, children - Adenoid cystic carcinoma

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Breast malignant, males, children

Carcinoma subtypes

Adenoid cystic carcinoma

Reviewer: Monika Roychowdhury, M.D. (see Reviewers page)
Revised: 10 April 2013, last major update March 2012
Copyright: (c) 2001-2013, PathologyOutlines.com, Inc.

General
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● Identical to salivary gland counterpart
● May be associated with microglandular adenosis (Am J Surg Pathol 2003;27:1052)

Epidemiology
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● Rare, 0.1% of breast carcinomas (Am J Surg 2002;183:646)
● Mean age 50-63 years, range 25-80 years
● 50% are sub-periareolar

Treatment and prognosis
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● Good to excellent prognosis (Breast Cancer Res Treat 2004;87:225)
● Recurs or metastasizes less than usual ductal carcinoma
● High rates of positive margins (Am J Clin Oncol 2010;33)
● Axillary nodal metastases are rare
● Treatment is excision with clear margins, possibly radiation, axillary dissection may not be necessary
● Prognosis better than that of adenoid cystic carcinoma of other localizations (Breast 2011 Dec 9 [Epub ahead of print])
● Existence of a dedifferentiated component of higher malignancy grade worsens the prognosis (Mod Pathol 2003;16:1265)

Case reports
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● 54 year old woman (UPMC Case #140)
● 55 year old woman (The Internet Journal of Pathology 2008;7(2))
● 61 year old woman with mammographic left breast mass (Case of the Week #271)
● 67 year old woman with mixed adenoid cystic and infiltrating ductal carcinoma of the breast (J Med Case Reports 2011;5:437)
● 71 year old woman (Int Semin Surg Oncol 2006;3:17)
● 75 year old woman (Arch Pathol Lab Med 2003;127:e436)
● 82 year old man (Int Surg 2006;91:234)
● Metastatic to kidney (Hum Pathol 2007;38:1425)

Gross description
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● 1-3 cm, well circumscribed, firm
● May have cystic cut surface

Micro description/grading
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● Identical to salivary gland counterpart; has cribriform, solid, trabecular or basaloid patterns
● Two types of cavities and two types of cells: (1) true glandular lumina lined by ductal epithelium (EMA+, keratin+) and (2) eosinophilic “cylinders” with basement membrane material lined by basal / myoepithelial-type cells (vimentin+)
● Microscopic infiltration and perineural invasion are common
● May have sebaceous differentiation (Arch Pathol Lab Med 1986;110:1045)

Micro images
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Low power

Cribriform pattern


Trabecular pattern

Solid, cribriform, tubular and trabecular patterns of small dark tumor cells
with scant cytoplasm and vesicular nuclei

Cribriform architecture in fibrous background

Lumina contain blue material or eosinophilic cylinders

High power

H&E and stains


Various images

Smooth muscle actin+

CK5/6, CK 8/18 and p63

c-kit/CD117+

H&E and stains - comparison with collagenous spherulosis

c-kit comparison with collagenous spherulosis

Mucicarmine

Case of the Week #271:

CD117+

AFIP Third Series:

Tumor nodule with central cysts and invasive elements above

13 year old boy with classic invasive growth pattern

Area with primarily cylindromatous component and rare glands (arrow)

Cytology description
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● Clusters of epithelial cells oriented around solid spheres of basement membrane material

Cytology images
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Various images

Monomorphic tumor cells around mucoid material

Positive stains
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Ductal epithelial cells:
● EMA
● Keratin
● c-kit/CD117 (Mod Pathol 2005;18:1623)

Basaloid/myoepithelial-type cells:
● p63
● S100
● Smooth muscle actin

Secretions in true lumina:
● PAS+ diastase resistant

Cribriform spaces:
● Alcian blue (Am J Clin Pathol 2005;124:733)

Negative stains
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● ER, PR (usually), HER2 (Mod Pathol 2005;18:1277)
● Calponin
● Smooth muscle myosin heavy chain

Cytogenetics description
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● MYB-NFIB fusion gene (t(6;9)(q22-23;p23-24)) has been found in adenoid cystic carcinomas of breast (J Pathol. 2012 Jan;226(1):84-96), making them a genomically distinct subgroup of triple-negative breast cancer

Differential diagnosis
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● Collagenous spherulosis: has one cell type, calponin+, smooth muscle myosin heavy chain+, c-kit/CD117- (Mod Pathol 2006;19:1351)
● Cribriform carcinoma: in situ or invasive, has only one cell type, ER+, PR+, p63-, c-kit/CD117-

Additional references
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● Am J Surg Pathol 1998;22:569, Hum Pathol 1987;18:1276 (grading), Mod Pathol 1996;9:215, Stanford University

Solid variant of adenoid cystic carcinoma

Terminology
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● Also called basaloid type

Features
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● Axillary nodal metastases in 2 of 6 cases (Am J Surg Pathol 2002;26:413)
● May have poorer prognosis than classic type (APMIS 1999;107:762)

Case reports
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● Merging with small cell carcinoma (Pathol Res Pract 2005;201:705)

Gross description
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● Solitary mass, mean 4 cm

Gross images
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Contributed by Dr. Semir Vranic, University of Sarajevo, Bosnia and Herzegovina

Micro description
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● Basaloid cells with moderate to marked nuclear atypia, often with 5+ mitotic figures/10 HPF

Micro images
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Solid pattern with inconspicuous cylindromatous elements at arrows (AFIP)

Contributed by Dr. Hind Nassar, Johns Hopkins Medical Center, Maryland (USA):



p63                             Actin

Contributed by Dr. Semir Vranic, University of Sarajevo, Bosnia and Herzegovina:

Core biopsy




Low power


Medium power




High power

Virtual slides
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Adenoid cystic carcinoma - solid/basaloid type

Positive stains
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● Keratin, basement membrane, vimentin

Negative stains
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● ER, PR

Differential diagnosis
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● Cylindroma: no atypia, no mitotic figures, no infiltration, no mucin (Am J Clin Pathol 2005;123:866)

End of Breast malignant, males, children > Carcinoma subtypes > Adenoid cystic carcinoma

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