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| [[Pancreatic neuroendocrine tumor case study one|Case#1]] | | [[Pancreatic neuroendocrine tumor case study one|Case#1]] |
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| __NOTOC__
| | ==Related chapters== |
| {{Pancreatic neuroendocrine tumor}}
| | * [[Pancreatic cancer]] |
| {{PleaseHelp}}
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| ==References==
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| {{Reflist|2}}
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| ==Genetics==
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| [[DNA]] [[mutation]] analysis in well-differentiated pancreatic neuroendocrine tumors identified four important findings:<ref name="Jiao2011">{{cite journal |author= Jiao, Y.; Shi, C.; Edil, B. H.; De Wilde, R. F.; Klimstra, D. S.; Maitra, A.; Schulick, R. D.; Tang, L. H.; Wolfgang, C. L.; Choti, M. A.; Velculescu, V. E.; Diaz Jr, L. A.; Vogelstein, B.; Kinzler, K. W.; Hruban, R. H.; Papadopoulos, N.|title= DAXX/ATRX, MEN1, and mTOR Pathway Genes Are Frequently Altered in Pancreatic Neuroendocrine Tumors|journal= Science |volume= 331|issue=6021 |pages=1199–1203|year=2011 |pmid= 21252315|doi= 10.1126/science.1200609}}</ref><ref name=" McKenna2014">{{cite journal |author= McKenna, L. R.; Edil, B. H. |title= Update on pancreatic neuroendocrine tumors |journal= Gland surgery |volume= 3|issue=4 |pages=258–275|year=2014 |pmid=25493258|doi= 10.3978/j.issn.2227-684X.2014.06.03}}</ref>
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| * as expected, the [[gene]]s mutated in NETs, [[MEN1]], [[ATRX]], [[DAXX]], [[TSC2]], [[PTEN (gene)|PTEN]] and [[PIK3CA]],<ref name="Jiao2011" /> are different from the mutated genes previously found in [[pancreatic cancer|pancreatic]] [[adenocarcinoma]].<ref name="Jones2008">{{cite pmid|18772397}}</ref><ref name="Harada2009">{{cite pmid|19077451}}</ref> | |
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| * one in six well-differentiated pancreatic NETs have mutations in [[mTOR]] pathway genes, such as [[TSC2]], [[PTEN (gene)|PTEN]] and [[PIK3CA]].<ref name="Jiao2011" /> The sequencing discovery might allow selection of which NETs would benefit from mTOR inhibition such as with [[everolimus]], but this awaits validation in a [[clinical trial]].
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| * mutations affecting a new cancer pathway involving [[ATRX]] and [[DAXX]] genes were found in about 40% of pancreatic NETs.<ref name="Jiao2011" /> The proteins encoded by ATRX and DAXX participate in [[chromatin]] remodeling of [[telomere]]s; these mutations are associated with a [[telomerase]]-independent maintenance mechanism termed ALT (alternative lengthening of telomeres) that results in abnormally long telomeric ends of [[chromosomes]].
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| * [[ATRX]]/[[DAXX]] and [[MEN1]] mutations were associated with a better [[prognosis]].<ref name="Jiao2011" />
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| ==References==
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| {{Reflist|2|refs=
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| <--This reference list is organized alphanumerically by arbitrary ref name-->
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| <ref name="Afinitor-PI">http://www.pharma.us.novartis.com/product/pi/pdf/afinitor.pdf</ref>
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| <ref name="ASCOPost20110515Evero">Everolimus Approved for Pancreatic Neuroendocrine Tumors. The ASCO Post. May 15, 2011, Volume 2, Issue 8 http://ascopost.com/articles/may-15-2011/everolimus-approved-for-pancreatic-neuroendocrine-tumors/</ref>
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| <ref name="Benson2010">Benson AB, Myerson RJ, and Sasson AR. Pancreatic, neuroendocrine GI, and adrenal cancers. Cancer Management: A Multidisciplinary Approach 13th edition 2010. ISBN 978-0-615-41824-7 Text is available electronically (but may require free registration) at http://www.cancernetwork.com/cancer-management/pancreatic/article/10165/1802606</ref>
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| <ref name="Burns2012">{{cite journal| author=Burns WR, Edil BH| title=Neuroendocrine pancreatic tumors: guidelines for management and update| journal=Current treatment options in oncology| date=March 2012 |volume=13| issue=1| pages=24–34| pmid=22198808| doi=10.1007/s11864-011-0172-2}}</ref>
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| <ref name="EU2010">{{cite news|url=http://www.genengnews.com/gen-news-highlights/pfizer-scores-new-approval-for-sutent-in-europe/81244326/ |title=Pfizer Scores New Approval for Sutent in Europe |date=2 Dec 2010 }}</ref>
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| <ref name="Grant2005">{{cite journal| author=Grant C | title=Insulinoma| journal= Best Practice & Research Clinical Gastroenterology | date=2005|volume=19 | issue=5| pages=783–798 | pmid=16253900| doi=10.1016/j.bpg.2005.05.008}}</ref>
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| <ref name="Heaphy2011">{{cite journal| author=Heaphy CM, De Wilde RF, Jiao Y, et al | date=2011| title=Altered Telomeres in Tumors with ATRX and DAXX Mutations| journal=Science |volume=333| issue=6041| pages= 425| doi=10.1126/science.1207313 | pmid=3174141 | pmid=21719641}}</ref>
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| <ref name="Jensen2008">{{cite journal| author=Jensen RT, Berna MJ, Bingham DB, Norton JA | title=Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies| journal= Cancer | date=2008|volume=113| issue=7 Suppl| pages= 1807–1843| pmid=18798544|pmc=2574000| doi=10.1002/cncr.23648}}</ref>
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| <ref name="Klimstra2010">The PanNET denomination is in line with current [[WHO]] guidelines. Historically, PanNETs have also been referred to by a variety of terms, and are still often called "islet cell tumors" or "pancreatic endocrine tumors". See: {{cite journal |author=Klimstra DS, Modlin IR, Coppola D, et al. |title=The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems |journal=Pancreas |volume=39 |issue=6 |pages=707–12 | date=August 2010 |pmid=20664470 |doi=10.1097/MPA.0b013e3181ec124e |url=http://www.seen.es/docs/apartados/470/The_Pathologic_Classification_of_Neuroendocrine.2.pdf}}</ref>
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| <ref name="NCCN_NET201501">{{cite web|title=Neuroendocrine tumors, NCCN Guidelines Version 1.2015|website=NCCN Guidelines|publisher=National Comprehensive Cancer Network, Inc. |url=http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf|accessdate=December 25, 2014|date=November 11, 2014}}</ref>
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| <ref name="NCIPET">National Cancer Institute. Cancer Drug Information. FDA Approval for Sunitinib Malate. Pancreatic Neuroendocrine Tumors http://www.cancer.gov/cancertopics/druginfo/fda-sunitinib-malate</ref>
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| <ref name=Oberg-2012>{{cite journal |author=Öberg K, Knigge U, Kwekkeboom D, Perren A |title=Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up |journal=Annals of Oncology: Official Journal of the European Society for Medical Oncology / ESMO |volume=23 Suppl 7 |issue= |pages=vii124–30 | date=October 2012 |pmid=22997445 |doi=10.1093/annonc/mds295 |url=http://annonc.oxfordjournals.org/content/23/suppl_7/vii124.long#T2}} ([http://annonc.oxfordjournals.org/content/23/suppl_7/vii124/T5.expansion.html Table 5] outlines the proposed TNM staging system for PanNETs.)</ref>
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| <ref name="PDQ">Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ) Health Professional Version. National Cancer Institute. March 7, 2014. [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0032521/can]</ref>
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| <ref name="Ramage2005">{{cite journal |author=Ramage JK, Davies AH, Ardill J, et al.|title=Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours |journal=Gut |volume=Suppl 4 |issue= suppl_4|pages=iv1–16 |date=Jun 2005 |series=54 |pmid=15888809|pmc=1867801|doi=10.1136/gut.2004.053314|url=http://gut.bmj.com/cgi/content/full/54/suppl_4/iv1}}</ref>
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| <ref name= "Raymond2011">{{cite journal | author = Raymond E, Dahan L, Raoul JL, et al. | year = 2011 | title = Sunitinib malate for the treatment of pancreatic neuroendocrine tumors | url = | journal = N Engl J Med | volume = 364 | issue = 6| pages = 501–13 | pmid = 21306237 | doi=10.1056/NEJMoa1003825}}</ref>
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| <ref name="Sutent-PI">http://labeling.pfizer.com/ShowLabeling.aspx?id=607</ref>
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| <ref name="Tejani2014">{{cite journal| author=Tejani MA, Saif MW (2014). "Pancreatic neuroendocrine tumors: Does chemotherapy work?". JOP : Journal of the pancreas 15 (2): 132–4. doi:10.6092/1590-8577/2301 (inactive 2014-12-26). PMID 24618436}}</ref>
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| ==External links== | | ==External links== |
| * {{DMOZ|Health/Conditions_and_Diseases/Cancer/Gastrointestinal/Pancreatic/Neuroendocrine/}} | | * {{DMOZ|Health/Conditions_and_Diseases/Cancer/Gastrointestinal/Pancreatic/Neuroendocrine/}} |
