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

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

^*Corresponding author for this work

Internal Medicine

IRCCS Ospedale San Raffaele
Universitätsspital Basel
Maimonides Biomedical Research Institute of Córdoba
University of Córdoba
Hospital Universitario Reina Sofía
Centro de Investigación Biomédica en Red (CIBER)
Sant'Andrea Hospital
Universidad Autónoma de Madrid
University of Manchester
University of Bern
University of Rome La Sapienza
Washington University School of Medicine in St. Louis
Beaujon Hospital
University of Exeter Medical School
Université Paris-Cité (Annecy)
Uppsala University
University Hospital Marburg

Research output: Contribution to journal › Article › Academic › peer-review

205 Citations (Scopus)

751 Downloads (Pure)

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.

Original language	English
Article number	e13318
Journal	Journal of Neuroendocrinology
Volume	35
Issue number	8
DOIs	https://doi.org/10.1111/jne.13318
Publication status	Published - Aug 2023

Bibliographical note

Publisher Copyright:
© 2023 The Authors. Journal of Neuroendocrinology published by John Wiley & Sons Ltd on behalf of British Society for Neuroendocrinology.

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SDG 3 Good Health and Well-being

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European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromesFinal published version, 2.61 MBLicence: CC BY-NC

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Hofland, J., Falconi, M., Christ, E., Castaño, J. P., Faggiano, A., Lamarca, A., Perren, A., Petrucci, S., Prasad, V., Ruszniewski, P., Thirlwell, C., Vullierme, M. P., Welin, S., & Bartsch, D. K. (2023). European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes. Journal of Neuroendocrinology, 35(8), Article e13318. https://doi.org/10.1111/jne.13318

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title = "European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes",

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.",

author = "Johannes Hofland and Massimo Falconi and Emanuel Christ and Casta{\~n}o, \{Justo P.\} and Antongiulio Faggiano and Angela Lamarca and Aurel Perren and Simona Petrucci and Vikas Prasad and Philippe Ruszniewski and Christina Thirlwell and Vullierme, \{Marie Pierre\} and Staffan Welin and Bartsch, \{Detlef K.\}",

note = "Publisher Copyright: {\textcopyright} 2023 The Authors. Journal of Neuroendocrinology published by John Wiley \& Sons Ltd on behalf of British Society for Neuroendocrinology.",

year = "2023",

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doi = "10.1111/jne.13318",

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Hofland, J, Falconi, M, Christ, E, Castaño, JP, Faggiano, A, Lamarca, A, Perren, A, Petrucci, S, Prasad, V, Ruszniewski, P, Thirlwell, C, Vullierme, MP, Welin, S & Bartsch, DK 2023, 'European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes', Journal of Neuroendocrinology, vol. 35, no. 8, e13318. https://doi.org/10.1111/jne.13318

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AU - Hofland, Johannes

AU - Falconi, Massimo

AU - Christ, Emanuel

AU - Castaño, Justo P.

AU - Faggiano, Antongiulio

AU - Lamarca, Angela

AU - Perren, Aurel

AU - Petrucci, Simona

AU - Prasad, Vikas

AU - Ruszniewski, Philippe

AU - Thirlwell, Christina

AU - Vullierme, Marie Pierre

AU - Welin, Staffan

AU - Bartsch, Detlef K.

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AB - 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.

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

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