Breast malignant, males, children
Carcinoma subtypes
Secretory carcinoma

Author: Gary Tozbikian, M.D.
Editorial Board Member Review: Emily S. Reisenbichler, M.D.
Deputy Editor in Chief: Debra Zynger, M.D.

Revised: 10 July 2018, last major update May 2018

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Secretory breast carcinoma

Cite this page: Tozbikian, G. Secretory carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantjuvenile.html. Accessed September 26th, 2018.
Definition / general
  • Rare subtype of low grade, translocation associated invasive breast carcinoma
  • Tumor with microcystic, solid and tubular architecture, composed of vacuolated tumor cells producing intracellular and extracellular secretions
  • Generally triple negative with basal-like phenotype (Mod Pathol 2012;25:567)
  • First described by McDivitt and Stewart in 1966 (JAMA 1966;195:388)
Essential features
  • Rare, < 1% of breast cancers
  • Most common primary pediatric breast cancer, but can occur at any age
  • Circumscribed margins, solid nests, cysts and gland formation with PAS+ intraluminal secretions
  • Tumor cells with abundant vacuolated or granular cytoplasm, low nuclear grade
  • Generally triple negative for estrogen receptor, progesterone receptor and HER2
  • Most tumors have translocation yielding ETV6-NTRK3 fusion gene
  • Associated with a favorable prognosis in younger patients
Terminology
  • Originally named juvenile breast carcinoma, the terminology was changed to secretory carcinoma after reports of the tumor occurring in adults
ICD-0 coding
Epidemiology
Clinical features
Radiology description
Radiology images

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Hypoechoic lesion on ultrasound

Case reports
Treatment
Clinical images

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Breast mass involving skin

Gross description
Microscopic (histologic) description
  • Well circumscribed with pushing borders but may be infiltrative at periphery
  • Central sclerosis may be observed
  • Architecture is usually microcystic, solid or tubular or admixture of all 3 patterns
  • Can display peripheral papillary architecture
  • Low grade cytologic atypia, bland uniform nuclei, low mitotic rate
  • Histologic hallmark are the presence of tumor cells with vacuolated, foamy cytoplasm and abundant intracellular and extracellular pale blue to dense pink secretions which are periodic acid Schiff (PAS) positive and diastase resistant (JAMA 1966;195:388, Mod Pathol 2012;25:567)
  • Often an in situ component (Mod Pathol 2009;22:291)
Microscopic (histologic) images

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Contributed by Gary Tozbikian, M.D.

Glands and solid nests

Tumor cells with vacuolated cytoplasm

Low nuclear grade, no mitoses, 40x


Secretory carcinoma 4x

Secretory carcinoma 10x

Secretory carcinoma 20x

Secretory carcinoma PAS-D 20x

Secretory carcinoma S100 20x


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Case of the Week #8


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H&E, PAS

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Microcystic glands with eosinophilic secretions

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Well differentiated but invasive glands

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PAS, S100, E-cadherin

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S100+

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S100, EMA, pan cytokeratin, ER

Positive stains
Molecular / cytogenetics description
Molecular / cytogenetics images

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Karyogram with t(12;15)(p13;q25)

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FISH with disrupted ETV6 gene

Differential diagnosis
  • Acinic cell carcinoma: cells with clear cytoplasm (hypernephroid pattern), expresses proteins of the salivary gland counterpart of acinic cell carcinoma (e.g. alpha-1-chymotrypsin, salivary gland amylase, lysozyme), lacks the t(12;15) translocation
  • Invasive ductal carcinoma, NOS type: lacks vacuolated cytoplasm and secretions, typically conventional Triple Negative Breast Cancer (TNBC) are high grade, not cytologically bland as in secretory carcinoma
  • Cystic hypersecretory hyperplasia: cysts containing abundant, homogenous intraluminal secretions resembling thyroid colloid, cells lining cysts are bland, flat or cuboidal/columnar cells lacking vacuolated / granular cytoplasm, presence of intact myoepithelial cell layer
  • Glycogen-rich carcinoma: PAS positive, diastase-sensitive secretions, can demonstrate any grade of nuclear atypia
  • Lipid-rich carcinoma: demonstrates Oil Red O positive cytoplasmic vacuoles, negative or only focal PAS positivity, can demonstrate any grade of nuclear atypia
Board review question #1
    This breast tumor is associated with which cytogenetic abnormality?


  1. 1q chromosomal gains and 16q chromosomal losses
  2. HER2 gene amplification
  3. t(12;15)(p13;q25), resulting in ETV6-NTRK3 fusion gene
  4. t(6;9)( q22-23;p23-24) resulting in fusion of MYB and NFIB
  5. TP53 mutation
Board review answer #1
C. t(12;15)(p13;q25), resulting in ETV6-NTRK3 fusion gene
Board review question #2
    What histological and immunophenotypic features are most likely to be observed in a breast secretory carcinoma?

  1. Abundant PAS+, diastase sensitive secretions
  2. High grade cytologic atypia with brisk mitotic activity
  3. Irregular clusters of epithelial cells floating in pools of extracellular mucin
  4. Low grade cytologic atypia, triple negative for ER, PR and HER2
  5. Negative for basal-like markers CK5 / 6 or CK14
Board review answer #2
D. Low grade cytologic atypia, triple negative for ER, PR and HER2