Pancreatic neuroendocrine tumor pathophysiology
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Differentiating Pancreatic neuroendocrine tumor from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Genetics
DNA mutation analysis in well-differentiated pancreatic neuroendocrine tumors identified four important findings:[1][2]
- as expected, the genes mutated in NETs, MEN1, ATRX, DAXX, TSC2, PTEN and PIK3CA,[1] are different from the mutated genes previously found in pancreatic adenocarcinoma.[3][4]
- one in six well-differentiated pancreatic NETs have mutations in mTOR pathway genes, such as TSC2, PTEN and PIK3CA.[1] 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.[1] The proteins encoded by ATRX and DAXX participate in chromatin remodeling of telomeres; 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.
References
- ↑ 1.0 1.1 1.2 1.3 1.4 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. (2011). "DAXX/ATRX, MEN1, and mTOR Pathway Genes Are Frequently Altered in Pancreatic Neuroendocrine Tumors". Science. 331 (6021): 1199–1203. doi:10.1126/science.1200609. PMID 21252315.
- ↑ McKenna, L. R.; Edil, B. H. (2014). "Update on pancreatic neuroendocrine tumors". Gland surgery. 3 (4): 258–275. doi:10.3978/j.issn.2227-684X.2014.06.03. PMID 25493258.
- ↑ PMID 18772397 (PMID 18772397)
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