Pancreatic neuroendocrine tumor: Difference between revisions

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	Pancreatic neuroendocrine tumor
	Classification and external resources
ICD-10	C25.4
eMedicine	med/

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Revision as of 19:21, 17 August 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Islet-cell carcinoma

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Pancreatic neuroendocrine tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Future or Investigational Therapies

Case Studies

Case#1

References

External links

Template:DMOZ

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 [[Pancreatic neuroendocrine tumor case study one|Case#1]]
-==Genetics==
-[[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>
-* 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>
-* 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]].
-* 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]].
-* [[ATRX]]/[[DAXX]] and [[MEN1]] mutations were associated with a better [[prognosis]].<ref name="Jiao2011" />
 ==References==


Pancreatic neuroendocrine tumor
Classification and external resources
ICD-10	C25.4
eMedicine	med/

Pancreatic neuroendocrine tumor: Difference between revisions

Revision as of 19:21, 17 August 2015

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