scholarly journals Alternative Lengthening of Telomeres in Primary Pancreatic Neuroendocrine Tumors Is Associated with Aggressive Clinical Behavior and Poor Survival

2016 ◽  
Vol 23 (6) ◽  
pp. 1598-1606 ◽  
Author(s):  
Joo Young Kim ◽  
Jacqueline A. Brosnan-Cashman ◽  
Soyeon An ◽  
Sung Joo Kim ◽  
Ki-Byung Song ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Pancreatic Neuroendocrine Tumors ◽  
Poor Survival ◽  
Alternative Lengthening Of Telomeres ◽  
Clinical Behavior ◽  
Alternative Lengthening ◽  
Aggressive Clinical Behavior

scholarly journals Alternative lengthening of telomeres and ATRX/DAXX loss can be reliably detected in FNAs of pancreatic neuroendocrine tumors

2017 ◽  
Vol 125 (7) ◽  
pp. 544-551 ◽  
Author(s):  
Christopher J. VandenBussche ◽  
Derek B. Allison ◽  
Mindy K. Graham ◽  
Vivek Charu ◽  
Anne Marie Lennon ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Pancreatic Neuroendocrine Tumors ◽  
Alternative Lengthening Of Telomeres ◽  
Alternative Lengthening

scholarly journals Alternative Lengthening of Telomeres (ALT) in Pancreatic Neuroendocrine Tumors: Ready for Prime-Time in Clinical Practice?

2021 ◽  
Vol 23 (9) ◽  
Author(s):  
Claudio Luchini ◽  
Rita T. Lawlor ◽  
Samantha Bersani ◽  
Caterina Vicentini ◽  
Gaetano Paolino ◽  
...  
Keyword(s):  
Clinical Practice ◽  
High Risk ◽  
Neuroendocrine Tumors ◽  
Tumor Biology ◽  
Specific Marker ◽  
Fish Analysis ◽  
Pancreatic Neuroendocrine Tumors ◽  
Alternative Lengthening Of Telomeres ◽  
Alternative Lengthening ◽  
Pancreatic Origin

Abstract Purpose of Review Alternative lengthening of telomeres (ALT) is a telomerase-independent mechanism used by some types of malignancies, including pancreatic neuroendocrine tumors, to overcome the issue of telomere shortening, thus supporting tumor growth and cell proliferation. This review is focused on the most important achievements and opportunities deriving from ALT assessment in PanNET onco-pathology, highlighting the most promising fields in which such biomarker could be implemented in clinical practice. Recent Findings In pancreatic neuroendocrine tumors (PanNET), ALT is strongly correlated with the mutational status of two chromatin remodeling genes, DAXX and ATRX. Recent advances in tumor biology permitted to uncover important roles of ALT in the landscape of PanNET, potentially relevant for introducing this biomarker into clinical practice. Indeed, ALT emerged as a reliable indicator of worse prognosis for PanNET, helping in clinical stratification and identification of “high-risk” patients. Furthermore, it is a very specific marker supporting the pancreatic origin of neuroendocrine neoplasms and can be used for improving the diagnostic workflow of patients presenting with neuroendocrine metastasis from unknown primary. The activation of this process can be determined by specific FISH analysis. Summary ALT should be introduced in clinical practice for identifying “high-risk” PanNET patients and improving their clinical management, and as a marker of pancreatic origin among neuroendocrine tumors.

CT Radiogenomic Characterization of the Alternative Lengthening of Telomeres Phenotype in Pancreatic Neuroendocrine Tumors

2018 ◽  
Vol 211 (5) ◽  
pp. 1020-1025 ◽  
Author(s):  
Jonathan M. McGovern ◽  
Aatur D. Singhi ◽  
Amir A. Borhani ◽  
Alessandro Furlan ◽  
Kevin M. McGrath ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Pancreatic Neuroendocrine Tumors ◽  
Alternative Lengthening Of Telomeres ◽  
Alternative Lengthening

scholarly journals Analysis of potential response predictors to capecitabine/temozolomide in metastatic pancreatic neuroendocrine tumors

2016 ◽  
Vol 23 (9) ◽  
pp. 759-767 ◽  
Author(s):  
M Cives ◽  
M Ghayouri ◽  
B Morse ◽  
M Brelsford ◽  
M Black ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Proliferative Activity ◽  
Dna Methyltransferase ◽  
Predictive Biomarkers ◽  
Pancreatic Neuroendocrine Tumors ◽  
Mgmt Expression ◽  
Methylguanine Dna Methyltransferase ◽  
Response Predictors ◽  
Alternative Lengthening ◽  
Pathway Activation

The capecitabine and temozolomide (CAPTEM) regimen is active in the treatment of metastatic pancreatic neuroendocrine tumors (pNETs), with response rates ranging from 30 to 70%. Small retrospective studies suggest that O6-methylguanine DNA methyltransferase (MGMT) deficiency predicts response to temozolomide. High tumor proliferative activity is also commonly perceived as a significant predictor of response to cytotoxic chemotherapy. It is unclear whether chromosomal instability (CIN), which correlates with alternative lengthening of telomeres (ALT), is a predictive factor. In this study, we evaluated 143 patients with advanced pNET who underwent treatment with CAPTEM for radiographic and biochemical response. MGMT expression (n=52), grade (n=128) and ALT activation (n=46) were investigated as potential predictive biomarkers. Treatment with CAPTEM was associated with an overall response rate (ORR) of 54% by RECIST 1.1. Response to CAPTEM was not influenced by MGMT expression, proliferative activity or ALT pathway activation. Based on these results, no biomarker-driven selection criteria for use of the CAPTEM regimen can be recommended at this time.

scholarly journals ATRX loss is an independent predictor of poor survival in pancreatic neuroendocrine tumors

2018 ◽  
Vol 82 ◽  
pp. 249-257 ◽  
Author(s):  
Angela Chou ◽  
Malinda Itchins ◽  
Philip R. de Reuver ◽  
Jennifer Arena ◽  
Adele Clarkson ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Independent Predictor ◽  
Pancreatic Neuroendocrine Tumors ◽  
Poor Survival
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