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Gastric neuroendocrine neoplasms

Nature Reviews Disease Primers volume 10, Article number: 25 (2024) Cite this article

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Abstract

Gastric neuroendocrine neoplasms (gNENs) display peculiar site-specific features among all NENs. Their incidence and prevalence have been rising in the past few decades. gNENs comprise gastric neuroendocrine carcinomas (gNECs) and gastric neuroendocrine tumours (gNETs), the latter further classified into three types. Type I anatype II gNETs are gastrin-dependent and develop in chronic atrophic gastritis and as part of Zollinger–Ellison syndrome within a multiple endocrine neoplasia type 1 syndrome (MEN1), respectively. Type III or sporadic gNETs develop in the absence of hypergastrinaemia and in the context of a near-normal or inflamed gastric mucosa. gNECs can also develop in the context of variable atrophic, relatively normal or inflamed gastric mucosa. Each gNEN type has different clinical characteristics and requires a different multidisciplinary approach in expert dedicated centres. Type I gNETs are managed mainly by endoscopy or surgery, whereas the treatment of type II gNETs largely depends on the management of the concomitant MEN1. Type III gNETs may require both locoregional approaches and systemic treatments; NECs are often metastatic and therefore require systemic treatment. Specific data regarding the systemic treatment of gNENs are lacking and are derived from the treatment of intestinal NETs and NECs. An enhanced understanding of molecular and clinical pathophysiology is needed to improve the management and outcomes of patients’ gNETs.

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Fig. 1: Epidemiology and prognosis of gastric neuroendocrine neoplasms.
Fig. 2: Pathophysiology of gastric neuroendocrine tumour.
Fig. 3: Different endoscopic aspects of gastric neuroendocrine neoplasms.
Fig. 4: Appearance of type III gastric neuroendocrine tumour.
Fig. 5: Functional imaging in gastric neuroendocrine neoplasms.
Fig. 6: Management algorithm for type I gastric neuroendocrine tumours.
Fig. 7: Management algorithm for type III gastric neuroendocrine neoplasms.

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Authors and Affiliations

  1. Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum - University of Bologna, Bologna, Italy

    Giuseppe Lamberti & Davide Campana

  2. Medical Oncology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy

    Giuseppe Lamberti & Davide Campana

  3. Department of Medical-Surgical Sciences and Translational Medicine, Sapienza University of Rome, Rome, Italy

    Francesco Panzuto

  4. Digestive Disease Unit, Sant’Andrea University Hospital, ENETS Center of Excellence, Rome, Italy

    Francesco Panzuto

  5. Department of Medicine 1, Friedrich-Alexander-University of Erlangen-Nürnberg, Erlangen, Germany

    Marianne Pavel

  6. National Centre for Neuroendocrine Tumours, ENETS Centre of Excellence, St. Vincent’s University Hospital, Dublin, Ireland

    Dermot O’Toole

  7. Trinity College Dublin, St. James Hospital, Dublin, Ireland

    Dermot O’Toole

  8. Nuclear Medicine, Alma Mater Studiorum University of Bologna, Bologna, Italy

    Valentina Ambrosini

  9. Nuclear Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy

    Valentina Ambrosini

  10. Pancreatic Surgery, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy

    Massimo Falconi

  11. Medicine Department, Universidad Complutense de Madrid, Madrid, Spain

    Rocio Garcia-Carbonero

  12. Oncology Department, Hospital Universitario 12 de Octubre, Imas12, Madrid, Spain

    Rocio Garcia-Carbonero

  13. Department of Clinical Oncology, AC Camargo Cancer Center, Sao Paulo, Brazil

    Rachel P. Riechelmann

  14. Section of Anatomic Pathology, Department of Life Sciences and Public Health, Università Cattolica del Sacro Cuore, Rome, Italy

    Guido Rindi

  15. Department of Woman and Child Health Sciences and Public Health, Anatomic Pathology Unit, Fondazione Policlinico Universitario A. Gemelli – IRCCS, ENETS Center of Excellence, Rome, Italy

    Guido Rindi

Authors
  1. Giuseppe Lamberti
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  2. Francesco Panzuto
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  3. Marianne Pavel
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  4. Dermot O’Toole
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  5. Valentina Ambrosini
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  6. Massimo Falconi
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  7. Rocio Garcia-Carbonero
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Contributions

Introduction (G.L., G.R. and D.C.); Epidemiology (F.P.); Mechanisms/pathophysiology (G.R.), Diagnosis, screening and prevention (G.L., M.P., D.O’T. and V.A.), Management (G.L., F.P., M.F., R.G.-C. and D.C.), Quality of life (R.P.R.), Outlook (G.L., G.R. and D.C.); overview of Primer (D.C. and G.R.). G.L. and F.P. contributed equally and are co-first authors; D.C. and G.R. contributed equally and are co-last authors.

Corresponding author

Correspondence to Davide Campana.

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Competing interests

M.P. reports honoraria for presentations from Novartis, IPSEN, AAA, Boehringer Ingelheim, MSD, Recordati, Lilly and Sanofi; honoraria for advisory board or consultancy from AAA, IPSEN, Riemser and Hutchmed; fees to institution clinical trials from Novartis, ITM, AAA and IPSEN. V.A. reports honoraria for presentations from ESMIT, EANM/ESMO, AAA, Cineca and Elma Academy outside the submitted work. G.R. reports personal fees from AAA (invited speaker, speaker bureau), Bracco Imaging (consultancy) and being a reviewer for a grant application for GIMBE Foundation (Gruppo Italiano Medicina Fondata sull’Evidenza). D.C. reports honoraria for presentations from AAA, Ipsen, Novartis and Esteve. All other authors declare no competing interests.

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Nature Reviews Disease Primers thanks Mark Pritchard, Christos Toumpanakis, Helge Waldum and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Lamberti, G., Panzuto, F., Pavel, M. et al. Gastric neuroendocrine neoplasms. Nat Rev Dis Primers 10, 25 (2024). https://doi.org/10.1038/s41572-024-00508-y

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