Basic science and translational implications of current knowledge on neuroendocrine tumors

Neuroendocrine tumors (NETs) are a diverse group of malignancies that can occur in various organs, with a notable prevalence in the lungs and gastrointestinal tract, which are the focus of this Review. Although NETs are rare in individual organs, their incidence has increased over recent decades, hi...

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Published in:The Journal of clinical investigation Vol. 135; no. 5; pp. 1 - 12
Main Authors: Fernandez-Cuesta, Lynnette, Alcala, Nicolas, Mathian, Emilie, Derks, Jules, Thirlwell, Chrissie, Dayton, Talya, Marinoni, Ilaria, Perren, Aurel, Walter, Thomas, Foll, Matthieu
Format: Journal Article
Language:English
Published: United States American Society for Clinical Investigation 03.03.2025
Subjects:
Animals
Biology
Cancer
Classification
Classification systems
Gastrointestinal tract
Human health and pathology
Humans
Hépatology and Gastroenterology
Life Sciences
Lungs
Malignancy
Medical prognosis
Metastases
Metastasis
Morphology
Mutation
Neuroendocrine tumors
Neuroendocrine Tumors - classification
Neuroendocrine Tumors - genetics
Neuroendocrine Tumors - immunology
Neuroendocrine Tumors - metabolism
Neuroendocrine Tumors - pathology
Neuroendocrine Tumors - therapy
Pancreas
Review
Risk factors
Therapeutic targets
Translational Research, Biomedical
Translational Science, Biomedical
Tumor Microenvironment
United States
ISSN:1558-8238, 0021-9738, 1558-8238
Online Access:Get full text
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Summary:Neuroendocrine tumors (NETs) are a diverse group of malignancies that can occur in various organs, with a notable prevalence in the lungs and gastrointestinal tract, which are the focus of this Review. Although NETs are rare in individual organs, their incidence has increased over recent decades, highlighting the urgent need for current classification systems to evolve by incorporating recent advances in the understanding of NET biology. Several omics studies have revealed molecular subtypes, which, when integrated into existing classification frameworks, may provide more clinically relevant insights for patients with NETs. This Review examines recent progress in elucidating the biology of NETs, with a particular emphasis on the tumor microenvironment and cells of origin. The existence of different cells of origin, which may contribute to distinct molecular groups, along with profiles of immune infiltration - despite being generally low - could explain the emergence of more aggressive cases and the potential for metastatic progression. Given the molecular heterogeneity of NETs and the diversity of their microenvironments and different cells of origin, there is an urgent need to develop morphomolecular classification systems. Such systems would make it possible to better characterize tumor progression, identify new therapeutic targets, and, ultimately, guide the development of personalized therapies.
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ISSN:1558-8238
0021-9738
1558-8238
DOI:10.1172/JCI186702