Compendium of Cancer Genome Aberrations

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BRST5:Secretory Carcinoma (view source)

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{{Under Construction}}
   
==Primary Author(s)*==
 
==Primary Author(s)*==
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==Epidemiology / Prevalence==
 
==Epidemiology / Prevalence==
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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>{{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> (Breast 2012;21:350 PMID: 22494666, J Surg Oncol 2016;113:721 PMID: 27040042)
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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>
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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> (JAMA 1966;195:388 PMID: 4285563)
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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>  
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Wide age range, bimodal age distribution with peaks in second and seventh decades<ref name=":0" /> (Breast 2012;21:350 PMID: 22494666, Diagn Histopathol 2020;26:51[C1] )
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Wide age range, bimodal age distribution with peaks in second and seventh decades <ref name=":0" />  
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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 )
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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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!Notes
 
!Notes
 
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|t(12;15)(p13;q25)||''ETV6::NTRK3''||der(15)||92% (PMID: 12450792)
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|t(12;15)(p13;q25)||''ETV6::NTRK3''||der(15)||92% <ref name=":2" />
 
|Yes
 
|Yes
 
|Yes
 
|Yes
 
|Yes
 
|Yes
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|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
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|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>
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. 2021 Apr;6(2):100072 <nowiki>PMID 33676294</nowiki>).
   
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==Familial Forms==
 
==Familial Forms==
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None
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==Additional Information==
 
==Additional Information==
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Put your text here
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==Links==
 
==Links==
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===EXAMPLE Book===
 
===EXAMPLE Book===
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#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 <nowiki>PMID 15963235</nowiki> 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.
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#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==
 
==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.
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