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

Research output: Contribution to journalArticlepeer-review

17 Scopus citations

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 languageEnglish
Article numbere13318
JournalJournal of Neuroendocrinology
Volume35
Issue number8
DOIs
StatePublished - Aug 2023

Keywords

  • ENETS
  • VIPoma
  • gastrinoma
  • guideline
  • insulinoma
  • neuroendocrine tumour

Fingerprint

Dive into the research topics of 'European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes'. Together they form a unique fingerprint.

Cite this