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

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

Johannes Hofland  1 , Massimo Falconi  2 , Emanuel Christ  3   4 , Justo P Castaño  5   6   7   8 , Antongiulio Faggiano  9 , Angela Lamarca  10   11 , Aurel Perren  12 , Simona Petrucci  13   14 , Vikas Prasad  15 , Philippe Ruszniewski  16 , Christina Thirlwell  17 , Marie-Pierre Vullierme  18 , Staffan Welin  19 , Detlef K Bartsch  20
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Free article

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

Johannes Hofland et al. J Neuroendocrinol. 2023 Aug.
Free article

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.

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References

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