Department of Internal Medicine, Section of Endocrinology, ENETS Centre of Excellence, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.
Pancreatic Surgery and Transplantation Unit, Pancreas Translational and Clinical Research Centre, ENETS Center of Excellence, San Raffaele Scientific Institute IRCCS, Milan, Italy.
J Neuroendocrinol. 2023 Aug;35(8):e13318. doi: 10.1111/jne.13318. Epub 2023 Aug 14.
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.
本 ENETS 指南旨在为临床医生提供有关胰腺神经内分泌肿瘤（NET）功能性综合征的诊断、治疗和随访的实用建议。NET 相关功能性综合征的定义为存在临床综合征，并伴有激素水平异常升高的生化证据。在胰腺 NET 患者中可能会遇到不同的激素综合征，包括胰岛素瘤、胃泌素瘤以及罕见的胰高血糖素瘤、血管活性肠肽瘤、促肾上腺皮质激素瘤、甲状旁腺素相关肽瘤、类癌综合征、甲状腺癌、生长激素释放激素瘤和生长抑素瘤。本文提供的建议侧重于在胰腺起源的、分化良好的 1-3 级、功能性 NET 中对个体激素综合征进行生化、遗传和影像学检查，以及对特定亚型在十二指肠中也进行治疗管理。
