胃泌素瘤
胰高血糖素瘤
胰岛素瘤
类癌综合征
神经内分泌肿瘤
医学
胰腺
内科学
多发性内分泌肿瘤
激素
类癌
胰腺神经内分泌肿瘤
内分泌学
胰多肽
胃肠病学
胃泌素
胰高血糖素
生物
生物化学
分泌物
基因
作者
Johannes Hofland,Massimo Falconi,Emanuel Christ,Justo P. Castaño,Antongiulio Faggiano,Ángela Lamarca,Aurel Perren,Simona Petrucci,Vikas Prasad,Philippe Ruszniewski,Christina Thirlwell,Marie‐Pierre Vullierme,Staffan Welin,Detlef K. Bartsch
摘要
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.
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