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Primary Author(s)*
Hui Chen, MD, PhD, The University of Texas MD Anderson Cancer Center
Morteza Seifi, PhD, University of Wisconsin
Cancer Category/Type
Breast Tumours / Epithelial tumours of the breast
Cancer Sub-Classification / Subtype
Rare and salivary gland-type tumours / Secretory carcinoma
Definition / Description of Disease
Secretory carcinoma is a low-grade tumor displaying pushing borders and areas of unequivocal stromal invasion. Tumors may show combinations of microcystic, solid and tubular patterns. The microcystic pattern is composed of irregular shaped small cysts lined with single layer of tumor cells and filled with eosinophilic secretions. The tubular pattern shows luminal eosinophil secretions. The microcystic and tubular patterns can mimic thyroid follicles and can merge into solid islands. Tumor cells are polygonal with granular eosinophilic to foamy cytoplasm. Tumor nuclei are slightly enlarged and regular in shape with inconspicuous nucleoli. Mitotic activity is rare.
Synonyms / Terminology
Synonyms: Juvenile breast carcinoma (historical)
Epidemiology / Prevalence
Rare, < 0.02% of all breast cancers[1][2] (Breast 2012;21:350 PMID: 22494666, J Surg Oncol 2016;113:721 PMID: 27040042)
Initially described in children; most common childhood breast cancer[3] (JAMA 1966;195:388 PMID: 4285563)
Wide age range, bimodal age distribution with peaks in second and seventh decades[1] (Breast 2012;21:350 PMID: 22494666, Diagn Histopathol 2020;26:51[C1] )
M:F = 1:6 to 1:31 (World J Surg Oncol 2005;3:35 PMID: 15963235, Mod Pathol 2012;25:567 PMID: 22157932, J Surg Oncol 2016;113:721 PMID: 27040042 , Ann Oncol 2000;11:1343 PMID: 11106125 )
Clinical Features
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| Signs and Symptoms | Well-circumscribed mobile masses |
| Laboratory Findings | Not applicable |
Sites of Involvement
The tumors are commonly seen in sub-areolar area.
Morphologic Features
Microcystic, solid and tubular patterns
Immunophenotype
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| Finding | Marker |
|---|---|
| Positive (universal) | S100, EMA, TRK |
| Positive (subset) | CEA (polyclonal), mammaglobin, SOX10 |
| Negative (universal) | ER, PR, and HER2 |
| Negative (subset) |
Chromosomal Rearrangements (Gene Fusions)
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| Chromosomal Rearrangement | Genes in Fusion (5’ or 3’ Segments) | Pathogenic Derivative | Prevalence | Diagnostic Significance (Yes, No or Unknown) | Prognostic Significance (Yes, No or Unknown) | Therapeutic Significance (Yes, No or Unknown) | Notes |
|---|---|---|---|---|---|---|---|
| t(12;15)(p13;q25) | ETV6::NTRK3 | der(15) | 92% (PMID: 12450792) | Yes | Yes | Yes | The ETV6::NTRK3 fusion is diagnostic of secretory carcinoma of breast in the appropriate morphology and clinical context (Cancer Cell. 2002 Nov;2(5):367-76. PMID: 12450792). This fusion is responsive to targeted therapy such as larotrectinib (Vitrakvi) and entrectinib (Rozlytrek) (ESMO Open
. 2021 Apr;6(2):100072 PMID 33676294). |
Individual Region Genomic Gain/Loss/LOH
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| Chr # | Gain / Loss / Amp / LOH | Minimal Region Genomic Coordinates [Genome Build] | Minimal Region Cytoband | Diagnostic Significance (Yes, No or Unknown) | Prognostic Significance (Yes, No or Unknown) | Therapeutic Significance (Yes, No or Unknown) | Notes |
|---|---|---|---|---|---|---|---|
| N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
Characteristic Chromosomal Patterns
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| Chromosomal Pattern | Diagnostic Significance (Yes, No or Unknown) | Prognostic Significance (Yes, No or Unknown) | Therapeutic Significance (Yes, No or Unknown) | Notes |
|---|---|---|---|---|
| N/A | N/A | N/A | N/A |
Gene Mutations (SNV/INDEL)
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| Gene; Genetic Alteration | Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other) | Prevalence (COSMIC / TCGA / Other) | Concomitant Mutations | Mutually Exclusive Mutations | Diagnostic Significance (Yes, No or Unknown) | Prognostic Significance (Yes, No or Unknown) | Therapeutic Significance (Yes, No or Unknown) | Notes |
|---|---|---|---|---|---|---|---|---|
| N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
Epigenomic Alterations
N/A
Genes and Main Pathways Involved
| Gene; Genetic Alteration | Pathway | Pathophysiologic Outcome |
|---|---|---|
| NTRK3 fusion; Activating mutations | Ras-Mek1 and PI3K-Akt pathways | Increased cell growth and proliferation |
Genetic Diagnostic Testing Methods
FISH, RT-PCR, RNAseq
Familial Forms
None
Additional Information
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Links
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References
- ↑ 1.0 1.1 Horowitz, David P.; et al. (2012-06). "Secretory carcinoma of the breast: results from the survival, epidemiology and end results database". Breast (Edinburgh, Scotland). 21 (3): 350–353. doi:10.1016/j.breast.2012.02.013. ISSN 1532-3080. PMID 22494666. Check date values in:
|date=(help) - ↑ Jacob, John Doromal; et al. (2016-06). "Rare breast cancer: 246 invasive secretory carcinomas from the National Cancer Data Base". Journal of Surgical Oncology. 113 (7): 721–725. doi:10.1002/jso.24241. ISSN 1096-9098. PMID 27040042. Check date values in:
|date=(help) - ↑ McDivitt, R. W.; et al. (1966-01-31). "Breast carcinoma in children". JAMA. 195 (5): 388–390. ISSN 0098-7484. PMID 4285563.
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EXAMPLE Book
- Arce, C; et al. (2005-06) Secretory carcinoma of the breast containing the ETV6-NTRK3 fusion gene in a male: case report and review of the literature. World J Surg Oncol. 17:3:35. doi: 10.1186/1477-7819-3-35. doi: 10.1186/1477-7819-3-35. PMC PMC1184104 PMID 15963235 Krings G, et al., (2019). Secretory carcinoma, in World Health Organization Classification of Tumours of Breast Tumours, Revised 5th edition. Allison KH, Brogi E, Ellis IO, Fox SB, Morris EA, Sahin A, Salgado R, Sapino A, Sasano H, Schnitt SJ, Sotiriou C, van Diest PJ, Editorial board expert members. IARC Press: Lyon, France, p146-148.
Notes
*Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.