European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes
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Authors
Hofland, J.Falconi, M.
Christ, E.
Castaño, J. P.
Faggiano, A.
Lamarca, Angela
Perren, A.
Petrucci, S.
Prasad, V.
Ruszniewski, P.
Thirlwell, C.
Vullierme, M. P.
Welin, S.
Bartsch, D. K.
Affiliation
Department of Internal Medicine, Section of Endocrinology, ENETS Centre of Excellence, Erasmus MC Cancer Institute, Rotterdam, The NetherlandsIssue Date
2023
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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.Citation
Hofland J, Falconi M, Christ E, Castaño JP, Faggiano A, Lamarca A, et al. European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes. Journal of neuroendocrinology. 2023 Jun 20:e13318. PubMed PMID: 37578384. Epub 2023/08/14. eng.Journal
Journal of NeuroendocrinologyDOI
10.1111/jne.13318PubMed ID
37578384Additional Links
https://dx.doi.org/10.1111/jne.13318Type
ArticleLanguage
enae974a485f413a2113503eed53cd6c53
10.1111/jne.13318
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