Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Apr;111(2):133-138.
doi: 10.1177/03008916251317128. Epub 2025 Feb 26.

Towards a better registration of neuroendocrine neoplasms: The results of the Italian retrospective population-based study

Annalisa Trama  1 , Francesco Cuccaro  2 , Domenico Damiani  3 , Alice Bernasconi  1 , Francesco Panzuto  4 , Nicola Fazio  5 , Simona Carone  6 , Maria Giovanna Burgio Lo Monaco  7 , Rossella Bruni  7 , Adele Caldarella  8 , Annalisa Roselli  8 , Barbara Cortini  8 , Rosalba Amodio  9 , Walter Mazzucco  9 , Silvia Leite  10 , Chiara Lupi  11 , Maddalena Baracco  12 , Eva Carpin  12 , Antonella Dal Cin  12 , Annarita Fiore  12 , Laura Memo  12 , Stefano Guzzinati  12 , Antonietta Alfia Maria Torrisi  13 , Antonina Torrisi  13 , Margherita Ferrante  13 , Maria Teresa Pesce  14 , Alessandra Sessa  14 , Antonietta Minichino  14 , Nadia Di Pascale  14 , Maria Francesca Vitale  15 , Mario Fusco  15 , Ilaria Girolami  3 , Massimo Milione  16 , Fabrizio Stracci  10   11   17
Affiliations

Towards a better registration of neuroendocrine neoplasms: The results of the Italian retrospective population-based study

Annalisa Trama et al. Tumori. 2025 Apr.

Abstract

Background: The increasing incidence, rapidly evolving classification, rarity and heterogeneity of neuroendocrine neoplasms (NENs) pose challenges to NEN registration including difficulty in distinguishing neuroendocrine carcinoma (NEC) and neuroendocrine tumours (NETs). Thus, in Italy a higher NEC incidence was reported. Focusing on gastroenteropancreatic (GEP) NEN, we aimed to review GEP NEN, and in particular cases of neuroendocrine carcinoma, not otherwise specified (NOS) and estimate the incidence of NEN, NET and NEC of the GEP.MethodsWe launched a pilot study examining cases of neuroendocrine carcinomas NOS (ICD-O3 code 8246) of GEP incidents in the years 2012-2020. Cancer registries (CRs) reviewed information included in the pathology report regarding differentiation and tumour cells proliferation to decide whether to confirm the case as neuroendocrine carcinoma NOS or register it as NET or NEC. After the review, we estimated the GEP NEN, NET and NEC incidence.

Results: Nine CRs contributed to the pilot study. After review, in all CRs, only 31% of GEP NOS neuroendocrine carcinomas were confirmed; 50% were recoded as NETs, and approximately 17% of cases were non-NENs. The IR of GEP NENs was 2.99/100,000, and the incidence of NETs was higher than that of NECs.

Conclusion: After the review, the incidence of GEP NEN, NET and NEC in the eight Italian CRs involved was comparable to that reported in other European countries.

Impact: Our results confirmed that heterogeneity of cancer registries in the registration of NEN requires collaborative work to define and promote a standard definition to be extended to all Italian registries.

Keywords: epidemiology; neuroendocrine neoplasms; population based cancer registries.

PubMed Disclaimer

Conflict of interest statement

Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

References

    1. Gatta G, Capocaccia R, Botta L, et al.. Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 2017; 18: 1022-1039. - PubMed
    1. Rossi RE, Massironi S. The increasing incidence of neuroendocrine neoplasms worldwide: Current knowledge and open issues. J Clin Med 2022; 11: 3794. - PMC - PubMed
    1. Fraenkel M, Kim M, Faggiano A, et al.. Incidence of gastroenteropancreatic neuroendocrine tumours: a systematic review of the literature. Endocr Relat Cancer 2014; 21: R153-63. - PubMed
    1. van der Zwan JM, Trama A, Otter R, et al.. Rare neuroendocrine tumours: results of the surveillance of rare cancers in Europe project. Eur J Cancer 2013; 49: 2565-2578. - PubMed
    1. Rindi G, Mete O, Uccella S, et al.. Overview of the 2022 WHO Classification of Neuroendocrine Neoplasms. Endocr Pathol 2022; 33: 115-154. - PubMed

MeSH terms

LinkOut - more resources