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

Johannes Hofland; Massimo Falconi; Emanuel Christ; Justo P Castaño; Antongiulio Faggiano; Angela Lamarca; Aurel Perren; Simona Petrucci; Vikas Prasad; Philippe Ruszniewski; Christina Thirlwell; Marie-Pierre Vullierme; Staffan Welin; Detlef K Bartsch

doi:10.1111/jne.13318

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

J Neuroendocrinol. 2023 Aug;35(8):e13318. doi: 10.1111/jne.13318. Epub 2023 Aug 14.

Authors

Johannes Hofland¹, Massimo Falconi², Emanuel Christ^{3

4}, Justo P Castaño^{5

6

7

8}, Antongiulio Faggiano⁹, Angela Lamarca^{10

11}, Aurel Perren¹², Simona Petrucci^{13

14}, Vikas Prasad¹⁵, Philippe Ruszniewski¹⁶, Christina Thirlwell¹⁷, Marie-Pierre Vullierme¹⁸, Staffan Welin¹⁹, Detlef K Bartsch²⁰

Affiliations

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.

PMID: 37578384
DOI: 10.1111/jne.13318

Abstract

This 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.

Keywords: ENETS; VIPoma; gastrinoma; guideline; insulinoma; neuroendocrine tumour.

MeSH terms

Gastrinoma* / diagnosis
Gastrinoma* / therapy
Glucagonoma* / diagnosis
Glucagonoma* / therapy
Humans
Insulinoma* / diagnosis
Insulinoma* / therapy
Neuroendocrine Tumors* / diagnosis
Neuroendocrine Tumors* / therapy
Pancreatic Neoplasms* / diagnosis
Pancreatic Neoplasms* / therapy