TY - JOUR
T1 - European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes
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.
N1 - Publisher Copyright:
© 2023 The Authors. Journal of Neuroendocrinology published by John Wiley & Sons Ltd on behalf of British Society for Neuroendocrinology.
PY - 2023/8
Y1 - 2023/8
N2 - 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.
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.
KW - ENETS
KW - VIPoma
KW - gastrinoma
KW - guideline
KW - insulinoma
KW - neuroendocrine tumour
UR - http://www.scopus.com/inward/record.url?scp=85168089420&partnerID=8YFLogxK
U2 - 10.1111/jne.13318
DO - 10.1111/jne.13318
M3 - Article
C2 - 37578384
AN - SCOPUS:85168089420
SN - 0953-8194
VL - 35
JO - Journal of Neuroendocrinology
JF - Journal of Neuroendocrinology
IS - 8
M1 - e13318
ER -