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#3007 An mRNA-based Classifier Identifies PanNETs with Different Clinicopathological Characteristics
Introduction: Pancreatic neuroendocrine tumors (PanNETs) are a heterogeneous group of neoplasms that varies from indolent to highly aggressive diseases. Previous studies have suggested alterations of ATRX/DAXX as biomarkers of dismal prognosis, yet inconclusive data (especially in the metastatic setting) prevent those biomarkers to be used as routine clinical tests.
Conference: 17th Annual ENETSConcerence (2020)
Presenting Author:
Authors: Vicentini C, Barbi S, Bersani S, Rusev B, Lawlor R,
Keywords: PanNET, gene expression profile, ATRX/DAXX mutations, patients’ stratification.,
Introduction: Pancreatic neuroendocrine tumors (PNETs) are rare and heterogeneous neoplasms with a 10-year survival rate less than 30%. Up to 43% of them are driven by inactivating mutations in ATRX or DAXX. These are important players at chromatin remodeling, that when mutated prone cancer cells to initiate the alternative lengthening of telomeres (ALT). ALT phenotype correlates with in situ loss of expression of the proteins ATRX or DAXX and can be corroborated by telomere-FISH.
Conference: 17th Annual ENETSConcerence (2020)
Presenting Author:
Authors: Gaspar T, Pinheiro J, Canberk S, İnce �, Tokat F,
Keywords: pancreas, pancreatic neuroendocrine tumors, PNETs, ATRX, DAXX, ALT, mosaic,
Introduction: Estimating prognosis of pancreatic neuroendocrine tumor (PNET) patients remains challenging. Mutation status of DAXX/ATRX/MEN1, histone modification patterns and immunohistochemistry for relevant transcription factors, including PDX1, were recently used to perform subtyping and distinguished two main types, A and B. These subtypes are linked to cell-of-origin and associated with clinical outcome.
Conference: 17th Annual ENETSConcerence (2020)
Presenting Author:
Authors: Boons G, Vandamme T, Ibrahim J, Schepers A, Roeyen G,
Keywords: Pancreatic Neuroendocrine Tumor, DNA Methylation, Prognostic Biomarker,
Introduction: MEN1, DAXX, ATRX, and PI3K/AKT/mTOR pathway genes are frequently mutated in pNETs. Sunitinib is approved and widely used in the metastatic setting but unfortunately, no validated predictive biomarker has been identified to guide therapy yet. The SUNEVO phase II trial of the Spanish Task Force Group for Neuroendocrine and Endocrine Tumours (GETNE) trial combined the pro-drug of EVO under SUN-induced hypoxic conditions.
Conference: 17th Annual ENETSConcerence (2020)
Presenting Author:
Authors: Santos M, Lanillos J, Lopez C, Alonso Gordoa T, Benavent M,
Keywords: pancreatic, neuroendocrine, mutations, correlation,
Introduction: Recent studies in pancreatic neuroendocrine tumors have identified mutations in DAXX/ATRX, MEN1, and genes involved in the phosphoinositide-3-kinase/Akt/mammalian target of rapamycin (PI3K-Akt-mTOR) pathway. However, these studies focused on abundant mutations.
Conference: 15th Annual ENETSConcerence (2018)
Presenting Author: Vandamme T
Authors: Vandamme T, Beyens M, Van Camp G, Boons G, Hofland L,
Keywords: pancreatic neuroendocrine tumors, PNET, genetics, tumor heterogeneity, hotspot mutations,