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An Insight on Functioning Pancreatic Neuroendocrine Neoplasms

Pancreatic neuroendocrine neoplasms (PanNENs) are rare neoplasms arising from islets of the Langerhans in the pancreas. They can be divided into two groups, based on peptide hormone secretion, functioning and nonfunctioning PanNENs. The first group is characterized by different secreted peptides cau...

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Autores principales: Bevere, Michele, Gkountakos, Anastasios, Martelli, Filippo Maria, Scarpa, Aldo, Luchini, Claudio, Simbolo, Michele
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9953748/
https://www.ncbi.nlm.nih.gov/pubmed/36830839
http://dx.doi.org/10.3390/biomedicines11020303
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author Bevere, Michele
Gkountakos, Anastasios
Martelli, Filippo Maria
Scarpa, Aldo
Luchini, Claudio
Simbolo, Michele
author_facet Bevere, Michele
Gkountakos, Anastasios
Martelli, Filippo Maria
Scarpa, Aldo
Luchini, Claudio
Simbolo, Michele
author_sort Bevere, Michele
collection PubMed
description Pancreatic neuroendocrine neoplasms (PanNENs) are rare neoplasms arising from islets of the Langerhans in the pancreas. They can be divided into two groups, based on peptide hormone secretion, functioning and nonfunctioning PanNENs. The first group is characterized by different secreted peptides causing specific syndromes and is further classified into subgroups: insulinoma, gastrinoma, glucagonoma, somatostatinoma, VIPoma and tumors producing serotonin and adrenocorticotrophic hormone. Conversely, the second group does not release peptides and is usually associated with a worse prognosis. Today, although the efforts to improve the therapeutic approaches, surgery remains the only curative treatment for patients with PanNENs. The development of high-throughput techniques has increased the molecular knowledge of PanNENs, thereby allowing us to understand better the molecular biology and potential therapeutic vulnerabilities of PanNENs. Although enormous advancements in therapeutic and molecular aspects of PanNENs have been achieved, there is poor knowledge about each subgroup of functioning PanNENs.Therefore, we believe that combining high-throughput platforms with new diagnostic tools will allow for the efficient characterization of the main differences among the subgroups of functioning PanNENs. In this narrative review, we summarize the current landscape regarding diagnosis, molecular profiling and treatment, and we discuss the future perspectives of functioning PanNENs.
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spelling pubmed-99537482023-02-25 An Insight on Functioning Pancreatic Neuroendocrine Neoplasms Bevere, Michele Gkountakos, Anastasios Martelli, Filippo Maria Scarpa, Aldo Luchini, Claudio Simbolo, Michele Biomedicines Review Pancreatic neuroendocrine neoplasms (PanNENs) are rare neoplasms arising from islets of the Langerhans in the pancreas. They can be divided into two groups, based on peptide hormone secretion, functioning and nonfunctioning PanNENs. The first group is characterized by different secreted peptides causing specific syndromes and is further classified into subgroups: insulinoma, gastrinoma, glucagonoma, somatostatinoma, VIPoma and tumors producing serotonin and adrenocorticotrophic hormone. Conversely, the second group does not release peptides and is usually associated with a worse prognosis. Today, although the efforts to improve the therapeutic approaches, surgery remains the only curative treatment for patients with PanNENs. The development of high-throughput techniques has increased the molecular knowledge of PanNENs, thereby allowing us to understand better the molecular biology and potential therapeutic vulnerabilities of PanNENs. Although enormous advancements in therapeutic and molecular aspects of PanNENs have been achieved, there is poor knowledge about each subgroup of functioning PanNENs.Therefore, we believe that combining high-throughput platforms with new diagnostic tools will allow for the efficient characterization of the main differences among the subgroups of functioning PanNENs. In this narrative review, we summarize the current landscape regarding diagnosis, molecular profiling and treatment, and we discuss the future perspectives of functioning PanNENs. MDPI 2023-01-21 /pmc/articles/PMC9953748/ /pubmed/36830839 http://dx.doi.org/10.3390/biomedicines11020303 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bevere, Michele
Gkountakos, Anastasios
Martelli, Filippo Maria
Scarpa, Aldo
Luchini, Claudio
Simbolo, Michele
An Insight on Functioning Pancreatic Neuroendocrine Neoplasms
title An Insight on Functioning Pancreatic Neuroendocrine Neoplasms
title_full An Insight on Functioning Pancreatic Neuroendocrine Neoplasms
title_fullStr An Insight on Functioning Pancreatic Neuroendocrine Neoplasms
title_full_unstemmed An Insight on Functioning Pancreatic Neuroendocrine Neoplasms
title_short An Insight on Functioning Pancreatic Neuroendocrine Neoplasms
title_sort insight on functioning pancreatic neuroendocrine neoplasms
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9953748/
https://www.ncbi.nlm.nih.gov/pubmed/36830839
http://dx.doi.org/10.3390/biomedicines11020303
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