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Breast malignant, males, children

Carcinoma subtypes

Secretory carcinoma


Reviewer: Monika Roychowdhury, M.D. (see Reviewers page)
Revised: 29 October 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

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 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)
● 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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Case of Week #8


Arising within radial scar


Intracytoplasmic lumina with secretions


AFIP images:
      
Intraductal component      Microcystic pattern below,
with microcystic features    solid apocrine pattern above


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


Various images


PAS+ diastase resistant secretions


Various immunostains


Figure 1: solid and cystic tumor with brown-yellow mucoid material in cysts
Figure 2: tumor cells are arranged in glandular pattern with eosinophilic secretions in lumen
Figure 3: focally are vacuolated secretory cells
Figure 4: secretions are PAS+ diastase resistant



Figure 1: FNA shows abundant, granular and vacuolated cytoplasm
Figure 2: firm white cut surface
Figure 3: tumor cells have abundant pale to pink or amphophilic cytoplasm and prominent nucleoli
Figure 4: secretions are PAS+


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, Int J Breast Cancer 2011;2011:704657

End of Breast malignant, males, children > Carcinoma subtypes > Secretory carcinoma


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