General
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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 by McDivitt and Stewart (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

Prognostic features
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• Excellent prognosis, although nodal metastases occur even in tumors < 2 cm (Ann Surg Oncol 2002;9:663, Cancer 1980;45:2404)
• 5 year survival near 100% in children; in women, poorer prognosis in women age 30+ years due to late recurrence and nodal metastases, but death due to disease is rare
• More aggressive in men (Ann Surg Oncol 2002;9:663)

Case reports
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• 7 year old girl with axillary nodal metastases (Ethiop Med J 2012;50:89)
• 17 year old boy with recurrent breast swelling (World J Surg Oncol 2004;2:17)
• 23 year old man with unilateral gynecomastia (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;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 (Arch Pathol Lab Med 2001;125:1372)
• 52 year old man with recurrent tumor at chest wall and lung metastases (World J Surg Oncol 2005;3:35)
• 52 year old woman with breast mass and two positive axillary lymph nodes (Case of the Week #8)
• 60 year old woman with tumor ulcerating through skin (Arch Pathol Lab Med 2006;130:1073)
• 61 year old woman with tumor exhibiting apocrine differentiation (Arch Pathol Lab Med 2006;130:e50)
• Young girl with axillary tumor (Am J Surg Pathol 2009;33:950)

Clinical images
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17 year old boy 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 and often near areola

Micro description
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• Sheet-like growth, usually circumscribed, pushing margins, occasional foci of infiltration (Stanford University - Secretory Carcinoma of the Breast)
• Tubuloalveolar and focally papillary formations composed of relatively bland cells with uniform, low-grade nuclei
• Abundant pale to eosinophilic, often vacuolated cytoplasm, but little pleomorphism
• Prominent nucleoli
• PAS+ diastase resistant eosinophilic secretions
• Also prominent hyalinization centrally, in situ component
• No atypia, no / rare mitotic figures
• May be difficult to differentiate in situ from invasive disease

Micro images
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H&E, PAS

Intracytoplasmic lumen, H&E

 

Courtesy of Dr. Mark R. Wick

 

Case of Week #8

   

Arising within radial scar

 

Intracytoplasmic lumina with secretions

Well-differentiated but invasive glands

PAS, S100, E-cadherin

S-100+

PAS+ diastase resistant secretions

S100, EMA, pan cytokeratin, ER

 

Various images

Intraductal component with microcystic features

Microcystic pattern below, solid apocrine pattern above

Secretion tends to shrink toward center of lumen, may have a scalloped border in larger microcysts

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 and mucicarmine
• Variable GCDFP-15 and CEA
• Secretions are Alcian blue+, PAS+
• Variable intranuclear or cytoplasmic p63 (Int J Surg Pathol 2012;20:367)

Negative stains
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• Usually ER, PR and HER2 (triple negative)

Molecular description
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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

Electron microscopy description
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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)

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, Arch Pathol Lab Med 1991;115:141, Mod Pathol 1994;7:475, Stanford University - Secretory Carcinoma of the Breast, Int J Breast Cancer 2011;2011:704657

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