ArticleClinical cancer research : an official journal of the American Association for Cancer Research2017
Alternative Lengthening of Telomeres and Loss of DAXX/ATRX Expression Predicts Metastatic Disease and Poor Survival in Patients with Pancreatic Neuroendocrine Tumors.
Article in Clinical cancer research : an official journal of the American Association for Cancer Research, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 140 papers, 5 of them syntheses that pooled it.
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Who cites it
140 citing papers in PubMed, 5 syntheses or guidelines pooled it, 239 citations in OpenAlex.
- Surgical Versus Nonsurgical Management of Pancreatic Neuroendocrine Tumors: A Systematic Review and Meta-Analysis.Annals of surgical oncology · 2025Pooled it
- Pooled it
- Prognostic Significance of Altered ATRX/DAXX Gene in Pancreatic Neuroendocrine Tumors: A Meta-Analysis.Frontiers in endocrinology · 2021Pooled it
- Prognostic factors for the outcome of nonfunctioning pancreatic neuroendocrine tumors in MEN1: a systematic review of literature.Endocrine-related cancer · 2020Pooled it
- Alternative lengthening of telomeres (ALT) influences survival in soft tissue sarcomas: a systematic review with meta-analysis.BMC cancer · 2019Pooled it
- Novel alternative tools for metastatic pheochromocytomas/paragangliomas prediction.Journal of endocrinological investigation · 2024Trial
- Sunitinib and Evofosfamide (TH-302) in Systemic Treatment-Naïve Patients with Grade 1/2 Metastatic Pancreatic Neuroendocrine Tumors: The GETNE-1408 Trial.The oncologist · 2021Trial
- Alternative lengthening of telomeres in small well-differentiated pancreatic neuroendocrine tumors: independent validation and cross-platform concordance.Virchows Archiv : an international journal of pathology · 2026Article
- A case of small pancreatic neuroendocrine tumor of less than 10 mm showing metastasis to multiple organs.Clinical journal of gastroenterology · 2026Article
- Review
- Real-World-Feasible Immunohistochemistry of ATRX, DAXX, and Menin Identifies a Subgroup of Non-Functioning Pancreatic Neuroendocrine Tumors with low Recurrence Risk to Guide De-Escalating Surveillance.Endocrine pathology · 2026Article
- Automated Radiomics Model for Preoperative Pancreatic Neuroendocrine Tumor Grade Prediction.Annals of surgical oncology · 2026Article
- Whole genome and exome sequencing of pancreatic neuroendocrine tumour to investigate PRRT response.Endocrine-related cancer · 2026Article
- Serotonin Expression and β-Cell Phenotype Are Associated With Indolent Behavior in Pancreatic Neuroendocrine Tumors With Sclerosing Morphology.Journal of surgical oncology · 2026Article
- Clinicopathological and molecular determinants of pancreatic neuroendocrine tumors: toward precision risk stratification.Journal of translational medicine · 2026Review
- Multi-omic profiles of neuroendocrine neoplasms of the pancreas: an integrated landscape.Molecular cancer · 2026Review
- Prognostic and Predictive Significance of Selected Gene Mutations in Pancreatic and Intestinal Neuroendocrine Tumors.International journal of molecular sciences · 2026Review
- ATRX loss in sarcomas is associated with dysregulated gene and transposable element expression, loss of DNA methylation, and worse survival.ESMO rare cancers · 2026Article
- Expression of ATRX, DAXX, PDX1, ARX, and somatostatin receptors in pancreatic neuroendocrine tumors: a clinicopathological study.Frontiers in endocrinology · 2026Article
- Concept of neuroendocrine neoplasms of all organs with a focus on grading, subtyping.Virchows Archiv : an international journal of pathology · 2026Review
80 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
17 authors at 3 institutions in 1 country.
Funding
Abstract
purposePancreatic neuroendocrine tumors (PanNET) are a heterogeneous group of neoplasms with increasing incidence and unpredictable behavior. Whole-exome sequencing has identified recurrent mutations in the genes DAXX and ATRX, which correlate with loss of protein expression and alternative lengthening of telomeres (ALT). Both ALT and DAXX/ATRX loss were initially reported to be associated with a favorable prognosis; however, recent studies suggest the contrary. Our aims were to assess the prevalence and prognostic significance of ALT and DAXX/ATRX in both primary and metastatic PanNETs. EXPERIMENTAL
designTelomere-specific FISH and DAXX/ATRX IHC was performed on a multi-institutional cohort of 321 patients with resected PanNET and 191 distant metastases from 52 patients. These results were correlated with clinicopathologic features, including disease-free survival (DFS) and disease-specific survival (DSS).
resultsThe prevalence of ALT and DAXX/ATRX loss in resected PanNETs was 31% and 26%, respectively, and associated with larger tumor size, higher WHO grade, lymph node metastasis, and distant metastasis (P < 0.001). The 5-year DFS and 10-year DSS of patients with ALT-positive and DAXX/ATRX-negative PanNETs were 40% and 50%, respectively, as compared with 96% and 89%, respectively, for wild-type PanNETs. Among distant metastases, ALT and DAXX/ATRX loss was 67% and 52%, respectively, and only occurred in the setting of an ALT-positive and DAXX/ATRX-negative primary PanNET. By multivariate analysis, both ALT and DAXX/ATRX loss were negative, independent prognostic factors for DFS.
conclusionsALT and DAXX/ATRX loss in PanNETs was associated with shorter DFS and DSS and likely plays a significant role in driving metastatic disease. Clin Cancer Res; 23(2); 600-9. ©2016 AACR.
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