European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1–G3

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Abstract

The aim of the present guidance paper was to update the previous ENETS guidelines on well‐differentiated gastric and duodenal neuroendocrine tumours (NETs), providing practical guidance for specialists in the diagnosis and management of gastroduodenal NETs. Type II gastric NETs, neuroendocrine carcinomas (NECs), and functioning duodenal NETs are not covered, since they will be discussed in other ENETS guidance papers.

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Most cited references 63

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Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States.

Arvind Dasari, Chan Shen, Daniel S. Halperin … (2017)

The incidence and prevalence of neuroendocrine tumors (NETs) are thought to be rising, but updated epidemiologic data are lacking.

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One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States.

James C Yao, Manal Hassan, Alexandria Phan … (2008)

PURPOSE Neuroendocrine tumors (NETs) are considered rare tumors and can produce a variety of hormones. In this study, we examined the epidemiology of and prognostic factors for NETs, because a thorough examination of neither had previously been performed. METHODS The Surveillance, Epidemiology, and End Results (SEER) Program registries were searched to identify NET cases from 1973 to 2004. Associated population data were used for incidence and prevalence analyses. Results We identified 35,618 patients with NETs. We observed a significant increase in the reported annual age-adjusted incidence of NETs from 1973 (1.09/100,000) to 2004 (5.25/100,000). Using the SEER 9 registry data, we estimated the 29-year limited-duration prevalence of NETs on January 1, 2004, to be 9,263. Also, the estimated 29-year limited-duration prevalence in the United States on that date was 103,312 cases (35/100,000). The most common primary tumor site varied by race, with the lung being the most common in white patients, and the rectum being the most common in Asian/Pacific Islander, American Indian/Alaskan Native, and African American patients. Additionally, survival duration varied by histologic grade. In multivariate analysis of patients with well-differentiated to moderately differentiated NETs, disease stage, primary tumor site, histologic grade, sex, race, age, and year of diagnosis were predictors of outcome (P < .001). CONCLUSION We observed increased reported incidence of NETs and increased survival durations over time, suggesting that NETs are more prevalent than previously reported. Clinicians need to be become familiar with the natural history and patterns of disease progression, which are characteristic of these tumors.

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A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal

Guido Rindi, David Klimstra, Behnoush Abedi-Ardekani … (2018)

The classification of neuroendocrine neoplasms (NENs) differs between organ systems and currently causes considerable confusion. A uniform classification framework for NENs at any anatomical location may reduce inconsistencies and contradictions among the various systems currently in use. The classification suggested here is intended to allow pathologists and clinicians to manage their patients with NENs consistently, while acknowledging organ-specific differences in classification criteria, tumor biology, and prognostic factors. The classification suggested is based on a consensus conference held at the International Agency for Research on Cancer (IARC) in November 2017 and subsequent discussion with additional experts. The key feature of the new classification is a distinction between differentiated neuroendocrine tumors (NETs), also designated carcinoid tumors in some systems, and poorly differentiated NECs, as they both share common expression of neuroendocrine markers. This dichotomous morphological subdivision into NETs and NECs is supported by genetic evidence at specific anatomic sites as well as clinical, epidemiologic, histologic, and prognostic differences. In many organ systems, NETs are graded as G1, G2, or G3 based on mitotic count and/or Ki-67 labeling index, and/or the presence of necrosis; NECs are considered high grade by definition. We believe this conceptual approach can form the basis for the next generation of NEN classifications and will allow more consistent taxonomy to understand how neoplasms from different organ systems inter-relate clinically and genetically.

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Author and article information

Contributors

Francesco Panzuto: (View ORCID Profile)

John Ramage: (View ORCID Profile)

D. Mark Pritchard: (View ORCID Profile)

Marie‐Louise F. van Velthuysen: (View ORCID Profile)

Anders Sundin: (View ORCID Profile)

Journal

Title: Journal of Neuroendocrinology

Abbreviated Title: J Neuroendocrinology

Publisher: Wiley

ISSN (Print): 0953-8194

ISSN (Electronic): 1365-2826

Publication date Created: August 2023

Publication date (Electronic): July 04 2023

Publication date (Print): August 2023

Volume: 35

Issue: 8

Affiliations

[1 ] Department of Medical‐Surgical Sciences and Translational Medicine, Digestive Disease Unit Sant'Andrea University Hospital, ENETS Center of Excellence, Sapienza University of Rome Rome Italy

[2 ] Department of Gastroenterology Hampshire Hospitals and ENETS Center, Kings Health Partners London London United Kingdom

[3 ] Department of Molecular and Clinical Cancer Medicine University of Liverpool Liverpool UK

[4 ] Department of Pathology, Erasmus MC, University Medical Center Rotterdam Rotterdam The Netherlands

[5 ] Department of Medicine University Medical Center Hamburg‐Eppendorf Hamburg Germany

[6 ] Department for General‐, Visceral‐, Thoracic‐ and Endocrine Surgery, Johannes‐Wesling‐Klinikum Minden University Hospital of the Ruhr‐University Bochum Bochum Germany

[7 ] Department of Surgical Sciences, Radiology & Molecular Imaging Uppsala University Uppsala Sweden

[8 ] Pancreas Translational and Clinical Research Center, Pancreatic Surgery Unit IRCCS San Raffaele Scientific Institute Milan Italy

[9 ] National Centre for Neuroendocrine Tumours, ENETS Centre of Excellence, St. Vincent's University Hospital Dublin Ireland

Article

DOI: 10.1111/jne.13306

PubMed ID: 37401795

SO-VID: 56ad4f3d-906f-41ab-8ed7-2ca410510b34

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http://creativecommons.org/licenses/by-nc-nd/4.0/

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European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1–G3

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Most cited references 63

Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States.

One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States.

A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal

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