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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
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==Cancer Category/Type==
==Cancer Category/Type==
Breast cancer
Breast Tumours / Epithelial tumours of the breast
==Cancer Sub-Classification / Subtype==
==Cancer Sub-Classification / Subtype==
Rare and salivary gland-type tumours / Secretory carcinoma
==Definition / Description of Disease==
==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 / Terminology==
Synonyms: Juvenile breast carcinoma (historical)
==Epidemiology / Prevalence==
==Epidemiology / Prevalence==
Rare, < 0.02% of all breast cancers. <ref name=":0">{{Cite journal|last=Horowitz|first=David P.|last2=Sharma|first2=Charu S.|last3=Connolly|first3=Eileen|last4=Gidea-Addeo|first4=Daniela|last5=Deutsch|first5=Israel|date=2012-06|title=Secretory carcinoma of the breast: results from the survival, epidemiology and end results database|url=https://pubmed.ncbi.nlm.nih.gov/22494666|journal=Breast (Edinburgh, Scotland)|volume=21|issue=3|pages=350–353|doi=10.1016/j.breast.2012.02.013|issn=1532-3080|pmid=22494666}}</ref><ref name=":1">{{Cite journal|last=Jacob|first=John Doromal|last2=Hodge|first2=Caitlin|last3=Franko|first3=Jan|last4=Pezzi|first4=Christopher M.|last5=Goldman|first5=Charles D.|last6=Klimberg|first6=Vicki Suzanne|date=2016-06|title=Rare breast cancer: 246 invasive secretory carcinomas from the National Cancer Data Base|url=https://pubmed.ncbi.nlm.nih.gov/27040042|journal=Journal of Surgical Oncology|volume=113|issue=7|pages=721–725|doi=10.1002/jso.24241|issn=1096-9098|pmid=27040042}}</ref>
Initially described in children; most common childhood breast cancer. <ref>{{Cite journal|last=McDivitt|first=R. W.|last2=Stewart|first2=F. W.|date=1966-01-31|title=Breast carcinoma in children|url=https://pubmed.ncbi.nlm.nih.gov/4285563|journal=JAMA|volume=195|issue=5|pages=388–390|issn=0098-7484|pmid=4285563}}</ref>
Wide age range, bimodal age distribution with peaks in second and seventh decades <ref name=":0" />
M:F = 1:6 to 1:31. <ref name=":1" /><ref>{{Cite journal|last=Arce|first=C.|last2=Cortes-Padilla|first2=D.|last3=Huntsman|first3=D. G.|last4=Miller|first4=M. A.|last5=Dueñnas-Gonzalez|first5=A.|last6=Alvarado|first6=A.|last7=Pérez|first7=V.|last8=Gallardo-Rincón|first8=D.|last9=Lara-Medina|first9=F.|date=2005-06-17|title=Secretory carcinoma of the breast containing the ETV6-NTRK3 fusion gene in a male: case report and review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/15963235|journal=World Journal of Surgical Oncology|volume=3|pages=35|doi=10.1186/1477-7819-3-35|issn=1477-7819|pmc=1184104|pmid=15963235}}</ref><ref>{{Cite journal|last=Li|first=Dali|last2=Xiao|first2=Xiuying|last3=Yang|first3=Wentao|last4=Shui|first4=Ruohong|last5=Tu|first5=Xiaoyu|last6=Lu|first6=Hongfen|last7=Shi|first7=Daren|date=2012-04|title=Secretory breast carcinoma: a clinicopathological and immunophenotypic study of 15 cases with a review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/22157932|journal=Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc|volume=25|issue=4|pages=567–575|doi=10.1038/modpathol.2011.190|issn=1530-0285|pmid=22157932}}</ref><ref>{{Cite journal|last=Herz|first=H.|last2=Cooke|first2=B.|last3=Goldstein|first3=D.|date=2000-10|title=Metastatic secretory breast cancer. Non-responsiveness to chemotherapy: case report and review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/11106125|journal=Annals of Oncology: Official Journal of the European Society for Medical Oncology|volume=11|issue=10|pages=1343–1347|doi=10.1023/a:1008387800525|issn=0923-7534|pmid=11106125}}</ref>
==Clinical Features==
==Clinical Features==
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{| class="wikitable"
|'''Signs and Symptoms'''
|Well-circumscribed mobile masses
|-
|'''Laboratory Findings'''
|Not applicable
|}
==Sites of Involvement==
==Sites of Involvement==
The tumors are commonly seen in sub-areolar area.
==Morphologic Features==
==Morphologic Features==
Microcystic, solid and tubular patterns
==Immunophenotype==
==Immunophenotype==
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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
! Finding !! Marker
!Finding!!Marker
|-
|-
|Positive (universal) || EXAMPLE CD1
|Positive (universal)||S100, EMA, TRK
|-
|-
|Positive (subset) || EXAMPLE CD2
|Positive (subset)||CEA (polyclonal), mammaglobin, SOX10
|-
|-
|Negative (universal) || EXAMPLE CD3
|Negative (universal)||ER, PR, and HER2
|-
|-
|Negative (subset) || EXAMPLE CD4
|Negative (subset)||
|}
|}
==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
! Chromosomal Rearrangement !! Genes in Fusion (5’ or 3’ Segments) !! Pathogenic Derivative !! Prevalence
!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
|-
|-
|EXAMPLE t(9;22)(q34;q11.2) || EXAMPLE 3'ABL1 / 5'BCR || EXAMPLE der(22) || EXAMPLE 5%
|t(12;15)(p13;q25)||''ETV6::NTRK3''||der(15)||92% <ref name=":2" />
|-
|Yes
|EXAMPLE t(8;21)(q22;q22) || EXAMPLE 5'RUNX1 / 3'RUNXT1 || EXAMPLE der(8) || EXAMPLE 5%
|Yes
|Yes
|The ''ETV6::NTRK3'' fusion is diagnostic of secretory carcinoma of breast in the appropriate morphology and clinical context. <ref name=":2">{{Cite journal|last=Tognon|first=Cristina|last2=Knezevich|first2=Stevan R.|last3=Huntsman|first3=David|last4=Roskelley|first4=Calvin D.|last5=Melnyk|first5=Natalya|last6=Mathers|first6=Joan A.|last7=Becker|first7=Laurence|last8=Carneiro|first8=Fatima|last9=MacPherson|first9=Nicol|date=2002-11|title=Expression of the ETV6-NTRK3 gene fusion as a primary event in human secretory breast carcinoma|url=https://pubmed.ncbi.nlm.nih.gov/12450792|journal=Cancer Cell|volume=2|issue=5|pages=367–376|doi=10.1016/s1535-6108(02)00180-0|issn=1535-6108|pmid=12450792}}</ref> This fusion is responsive to targeted therapy such as larotrectinib (Vitrakvi) and entrectinib (Rozlytrek). <ref>{{Cite journal|last=Krebs|first=M. G.|last2=Blay|first2=J.-Y.|last3=Le Tourneau|first3=C.|last4=Hong|first4=D.|last5=Veronese|first5=L.|last6=Antoniou|first6=M.|last7=Bennett|first7=I.|date=2021-04|title=Intrapatient comparisons of efficacy in a single-arm trial of entrectinib in tumour-agnostic indications|url=https://pubmed.ncbi.nlm.nih.gov/33676294|journal=ESMO open|volume=6|issue=2|pages=100072|doi=10.1016/j.esmoop.2021.100072|issn=2059-7029|pmc=8103537|pmid=33676294}}</ref>
|}
|}
==Characteristic Chromosomal Aberrations / Patterns==
==Individual Region Genomic Gain/Loss/LOH==
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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
! Chromosome Number !! Gain/Loss/Amp/LOH !! Region
!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
|-
|-
|EXAMPLE 8 || EXAMPLE Gain || EXAMPLE chr8:0-1000000
|N/A
|N/A
|N/A
|N/A
|N/A
|N/A
|N/A
|N/A
|-
|-
|EXAMPLE 7 || EXAMPLE Loss || EXAMPLE chr7:0-1000000
|
|}
|
|
==Gene Mutations (SNV/INDEL)==
|
|
|
|
|
|}
==Characteristic Chromosomal Patterns==
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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
! Gene !! Mutation !! Oncogene/Tumor Suppressor/Other !! Presumed Mechanism (LOF/GOF/Other; Driver/Passenger) !! Prevalence (COSMIC/TCGA/Other)
!Chromosomal Pattern
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|-
| EXAMPLE TP53 || EXAMPLE R273H || EXAMPLE Tumor Suppressor || EXAMPLE LOF || EXAMPLE 20%
|N/A
|}
|N/A
|N/A
|N/A
|
|}
==Gene Mutations (SNV/INDEL)==
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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
! Type !! Gene/Region/Other
!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
|-
|-
| Concomitant Mutations || EXAMPLE IDH1 R123H
|N/A
|-
|N/A
| Secondary Mutations || EXAMPLE Trisomy 7
|N/A
|-
|N/A
|Mutually Exclusive || EXAMPLE EGFR Amplification
|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.
==Epigenomics (Methylation)==
==Epigenomic Alterations==
N/A
==Genes and Main Pathways Involved==
==Genes and Main Pathways Involved==
<br />
{| class="wikitable sortable"
==Diagnostic Testing Methods==
|-
!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
|-
|NTRK3 fusion; Activating mutations
|Ras-Mek1 and PI3K-Akt pathways
|Increased cell growth and proliferation
|-
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|-
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|}
==Genetic Diagnostic Testing Methods==
FISH, RT-PCR, RNAseq
==Familial Forms==
==Familial Forms==
<br />
==Other Information==
==Additional Information==
<br />
==Links==
==Links==
Put your links here
Put your text placeholder here (use "Link" icon at top of page)
==References==
==References==
<references />
(use "Cite" icon at top of page)
===EXAMPLE Book===
#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.
#Arber DA, et al., (2008). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4thedition.Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, Editors. IARC Press: Lyon, France, p117-118.
== Notes ==
==Notes==
<nowiki>*</nowiki>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.
<nowiki>*</nowiki>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.