Breast – Malignant, Males, Children

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Breast-malignant, males, children

Secretory carcinoma

Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.

Reviewer: Daniel Visscher, M.D., University of Michigan Hospitals, February 2009 (see Reviewers page)

Revised: 22 November 2010

Last major update: September 2009

Definition

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● Tumor cells with granular eosinophilic cytoplasm and prominent intracellular and extracellular milk-like secretion

● First described in 1966 as juvenile carcinoma (JAMA 1966;195:388)

● A type of basal-like carcinoma (triple negative with expression of basal cytokeratins, Mod Pathol 2009;22:291)

Terminology

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● Also called juvenile carcinoma

Epidemiology

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● Rare, usually children (most common form of breast carcinoma in children) but can occur in adults

● Rare in males

Treatment and prognosis

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● Excellent prognosis, although nodal metastases occur even in tumors < 2 cm (Ann Surg Oncol 2002;9:663)

● 5 year survival near 100% in children, less favorable in women age 30+ years due to late recurrence and nodal metastases, but death due to disease is rare

Case reports

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● Young girl with axillary tumor (Am J Surg Pathol 2009;33:950)

● 17 year old man (World J Surg Oncol 2004;2:17)

● 23 year old man (Am J Surg Pathol 1988;12:150)

● 27 year old woman with recurrent tumor after mastectomy (Am J Surg Pathol 1993;17:715)

● 33 year old woman with positive sentinel node (World J Surg Oncol 2006 Dec 6;4:88)

● 40 year old woman with tumor arising within radial scar (Indian J Pathol Microbiol 2009;52:83)

● 46 year old woman with axillary tumor (Archives 2001;125:1372)

● 52 year old man (World J Surg Oncol 2005;3:35)

● 52 year old woman (Case of the week #8)

● 60 year old woman (Archives 2006;130:1073)

● 61 year old woman with tumor exhibiting apocrine differentiation (Archives 2006;130:e50)

Clinical images

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Young man with breast mass and skin involvement

7 year old Ethiopian girl with breast mass present for 2 years

Gross description

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● Small, well circumscribed, often near areola

Microscopic description

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● Tubuloalveolar and focally papillary formations composed of relatively bland cells

● Abundant pale to eosinophilic, often vacuolated cytoplasm but little pleomorphism

● Prominent nucleoli

● PAS+ diastase resistant eosinophilic secretions

● Also pushing margins, prominent hyalinization centrally

● No atypia, no/rare mitotic figures

● May be difficult to differentiate in situ from invasive disease

Micro images

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Medium power High power

PAS+ diastase resistant secretions

Intracytoplasmic lumina with secretions

Arising within radial scar

Various images

Fig 1: solid and cystic tumor with brown-yellow mucoid material in cysts

Fig 2: tumor cells are arranged in glandular pattern with eosinophilic secretions in lumen

Fig 3: focally are vacuolated secretory cells

Fig 4: secretions are PAS+ diastase resistant

Fig 1: FNA shows abundant, granular and vacuolated cytoplasm

Fig 2: firm white cut surface

Fig 3: tumor cells have abundant pale to pink or amphophilic cytoplasm and prominent nucleoli

Fig 4: secretions are PAS+

AFIP images:

Intraductal component with Secretion tends to shrink toward

microcystic features center of lumen, may have a

scalloped border in larger microcysts

Microcystic pattern below, solid apocrine pattern above

Cytology description

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● Cohesive sheets of monotonous cells with intracytoplasmic vacuoles, round nuclei and small nucleoli (Diagn Cytopathol 2005;32:47)

Positive stains

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● Keratin

● EMA

● S100

● Alpha-lactalbumin

● Variable GCDFP-15 and CEA

● Secretions are Alcian blue+, PAS+

Negative stains

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● Usually ER, PR and HER2 (triple negative)

Electron microscopy

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● Numerous membrane-bound intracytoplasmic secretory vacuoles

Electron microscopy images

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Large irregular cytoplasmic vacuoles lined by villi (AFIP)

Molecular / cytogenetics

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● Usually t(12;15)(p13;q25), producing ETV6-NTRK3 fusion gene that is also seen in cellular mesoblastic nephroma and infantile fibrosarcoma (Hum Pathol 2003;34:1299, Semin Cancer Biol 2005;15:215, Genes Chromosomes Cancer 2004;40:152, AtlasGeneticsOncology.org)

Molecular images

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FISH shows t(12;15) t(12;15) in 6 year old girl

Differential diagnosis

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● Acinic cell carcinoma - no t(12;15) (Histopathology 2008;52:840)

Additional references

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● Am J Surg Pathol 1980;4:465 (adults), Archives 1991;115:141, Mod Pathol 1994;7:475, Stanford University

End of Breast – Malignant, Males, Children > Secretory carcinoma

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