Neuroendocrine Tumor Epidemiology and Therapeutics

Neuroendocrine tumours (NETs) encompass a heterogeneous group of neoplasms arising from hormone‐producing cells dispersed throughout the body, most commonly in the gastroenteropancreatic system and bronchopulmonary tract. Over recent decades, global incidence and prevalence have risen markedly, with enhanced detection of small, indolent lesions contributing to increasing rates, particularly in rectal and pancreatic sites. Advances in classification now distinguish well-differentiated NETs from poorly differentiated neuroendocrine carcinomas, guiding prognostic assessment based on factors such as mitotic index and Ki-67 labelling. Therapeutic strategies have evolved from surgical resection towards multimodal regimens that include somatostatin analogues, targeted molecular inhibitors, peptide receptor radionuclide therapy (PRRT) and combination approaches. The growing integration of genomic profiling has revealed distinct molecular subtypes with treatment implications, and novel biomarkers and predictive tools are refining personalised management for patients worldwide.

Research from Nature Portfolio

Comprehensive genomic and transcriptomic analyses of pulmonary large-cell neuroendocrine carcinomas have delineated two molecular subgroups defined by alterations in TP53, STK11/KEAP1 or RB1, each exhibiting divergent neuroendocrine marker expression and immune pathway activation. This stratification refines diagnostic frameworks, underscores the importance of distinguishing these subtypes from small-cell lung cancer, and opens avenues for targeted kinase inhibitors or immunomodulatory therapies tailored to each molecular profile.

Neuroendocrine Tumor Epidemiology and Therapeutics publication trend

The graph below shows the total number of articles in neuroendocrine tumor epidemiology and therapeutics across all publications each year (not limited to Nature Index journals).

Technical terms

Gastroenteropancreatic neuroendocrine tumours (GEP-NETs): Neoplasms arising from neuroendocrine cells in the gastrointestinal tract and pancreas.

Progression-free survival (PFS): Time during and after treatment in which a patient’s disease does not worsen.

Somatostatin analogue: Synthetic peptide that mimics the inhibitory hormone somatostatin to control hormone secretion and tumour growth.

Peptide receptor radionuclide therapy (PRRT): Targeted radiotherapy that delivers radiation directly to neuroendocrine tumour cells via radiolabelled peptides.

Nomogram: Statistical tool that integrates multiple prognostic variables to predict individual patient outcomes.

Large-cell neuroendocrine carcinoma (LCNEC): A high-grade form of lung neuroendocrine tumour characterised by large cells and aggressive behaviour.

References

1. Safety and antitumor activity of metformin plus lanreotide in patients with advanced gastro-intestinal or lung neuroendocrine tumors: the phase Ib trial MetNET2. Journal of Hematology & Oncology (2023).
2. A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Modern Pathology (2018).
3. The joint IAEA, EANM, and SNMMI practical guidance on peptide receptor radionuclide therapy (PRRNT) in neuroendocrine tumours. European Journal of Nuclear Medicine and Molecular Imaging (2013).
4. Integrative genomic profiling of large-cell neuroendocrine carcinomas reveals distinct subtypes of high-grade neuroendocrine lung tumors. Nature Communications (2018).
5. Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States. JAMA Network Open (2021).
6. Anti-tumour effects of lanreotide for pancreatic and intestinal neuroendocrine tumours: the CLARINET open-label extension study. Endocrine Related Cancer (2016).