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.

European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes / Hofland, Johannes; Falconi, Massimo; Christ, Emanuel; Castaño, Justo P; Faggiano, Antongiulio; Lamarca, Angela; Perren, Aurel; Petrucci, Simona; Prasad, Vikas; Ruszniewski, Philippe; Thirlwell, Christina; Vullierme, Marie-Pierre; Welin, Staffan; Bartsch, Detlef K. - In: JOURNAL OF NEUROENDOCRINOLOGY. - ISSN 1365-2826. - 35:8(2023), pp. 1-19. [10.1111/jne.13318]

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

Faggiano, Antongiulio;Lamarca, Angela;Petrucci, Simona;
2023

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.
2023
enets; vipoma; gastrinoma; guideline; insulinoma; neuroendocrine tumour
01 Pubblicazione su rivista::01a Articolo in rivista
European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes / Hofland, Johannes; Falconi, Massimo; Christ, Emanuel; Castaño, Justo P; Faggiano, Antongiulio; Lamarca, Angela; Perren, Aurel; Petrucci, Simona; Prasad, Vikas; Ruszniewski, Philippe; Thirlwell, Christina; Vullierme, Marie-Pierre; Welin, Staffan; Bartsch, Detlef K. - In: JOURNAL OF NEUROENDOCRINOLOGY. - ISSN 1365-2826. - 35:8(2023), pp. 1-19. [10.1111/jne.13318]
File allegati a questo prodotto
File Dimensione Formato  
Hofland_European-Neuroendocrine-Tumor-Society_2023.pdf

accesso aperto

Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Creative commons
Dimensione 2.65 MB
Formato Adobe PDF
2.65 MB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1686370
Citazioni
  • ???jsp.display-item.citation.pmc??? 26
  • Scopus 47
  • ???jsp.display-item.citation.isi??? 51
social impact