European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes

Author:

Hofland Johannes1ORCID,Falconi Massimo2,Christ Emanuel34,Castaño Justo P.5678,Faggiano Antongiulio9,Lamarca Angela1011,Perren Aurel12,Petrucci Simona1314,Prasad Vikas15,Ruszniewski Philippe16,Thirlwell Christina17,Vullierme Marie‐Pierre18ORCID,Welin Staffan19,Bartsch Detlef K.20

Affiliation:

1. Department of Internal Medicine, Section of Endocrinology, ENETS Centre of Excellence Erasmus MC Cancer Institute Rotterdam The Netherlands

2. Pancreatic Surgery and Transplantation Unit, Pancreas Translational and Clinical Research Centre ENETS Center of Excellence, San Raffaele Scientific Institute IRCCS Milan Italy

3. ENETS Centre of Excellence for Neuroendocrine and Endocrine Tumours University Hospital Basel Basel Switzerland

4. Department of Endocrinology, Diabetology and Metabolism University Hospital Basel Basel Switzerland

5. Maimonides Biomedical Research Institute of Córdoba Córdoba Spain

6. Department of Cell Biology, Physiology, and Immunology University of Córdoba Córdoba Spain

7. Hospital Universitario Reina Sofía Córdoba Spain

8. Centro de Investigación Biomédica en Red de Fisiopatología de la Obesidad y Nutrición (CIBERobn) Córdoba Spain

9. Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, ENETS Centre of Excellence Sapienza University of Rome Rome Italy

10. Department of Oncology – OncoHealth Institute Fundación Jiménez Díaz University Hospital Madrid Spain

11. Department of Medical Oncology, The Christie NHS Foundation, Manchester, Division of Cancer Sciences University of Manchester Manchester UK

12. Institute of Pathology University of Bern Bern Switzerland

13. Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology Sapienza University of Rome Rome Italy

14. Medical Genetics and Advanced Cell Diagnostics Unit Sant Andrea University Hospital Rome Italy

15. Division of Nuclear Medicine, Mallinckrodt Institute of Radiology Washington University in St. Louis St. Louis Missouri USA

16. Department of Pancreatology, FHU MOSAIC Université Paris Cité – APHP Hôpital Beaujon Clichy France

17. University of Exeter Medical School, University of Exeter Exeter UK

18. Centre Hospitalier Annecy Genevois (CHANGE) Université Paris‐Cité, Praticien Hospitalier Imagerie Médicale Annecy France

19. Department of Endocrine Oncology Uppsala University Hospital Uppsala Sweden

20. Department of Visceral, Thoracic and Vascular Surgery Phillips‐University Marburg and University Hospital Gießen Marburg GmbH Marburg Germany

Abstract

AbstractThis ENETS guidance paper aims to provide practical advice to clinicians for the diagnosis, treatment and follow‐up of functioning syndromes in pancreatic neuroendocrine tumours (NET). A NET‐associated functioning syndrome is defined by the presence of a clinical syndrome combined with biochemical evidence of inappropriately elevated hormonal levels. Different hormonal syndromes can be encountered in pancreatic NET patients, including insulinoma, gastrinoma as well as the rare glucagonoma, VIPoma, ACTHoma, PTHrPoma, carcinoid syndrome, calcitoninoma, GHRHoma and somatostatinoma. The recommendations provided in this paper focus on the biochemical, genetic and imaging work‐up as well as therapeutic management of the individual hormonal syndromes in well‐differentiated, grade 1‐3, functioning NET with the primary tumour originating in the pancreas, and for specific subtypes also in the duodenum.

Publisher

Wiley

Subject

Cellular and Molecular Neuroscience,Endocrine and Autonomic Systems,Endocrinology,Endocrinology, Diabetes and Metabolism

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