Zandee Wouter T, Kamp Kimberly, van Adrichem Roxanne C, Feelders Richard A, de Herder Wouter W
Department of Internal MedicineSector Endocrinology, ENETS Centre of Excellence, Erasmus MC, Rotterdam, the Netherlands
Department of Internal MedicineSector Endocrinology, ENETS Centre of Excellence, Erasmus MC, Rotterdam, the Netherlands.
Endocr Relat Cancer. 2017 Jul;24(7):R261-R274. doi: 10.1530/ERC-16-0538. Epub 2017 May 8.
The treatment of hormone hypersecretory syndromes caused by neuroendocrine tumors (NETs) can be a major challenge. NETs originating from the small intestine often secrete serotonin causing flushing, diarrhea and valve fibrosis, leading to dehydration or heart failure in severe cases. NETs from the pancreas can secrete a wider variety of hormones, like insulin, glucagon and gastrin leading to distinct clinical syndromes. Historically mortality in patients with functioning NETs was high due to the complications caused by the hypersecretion of hormones. This has been reduced with several drugs: proton-pump inhibitors decrease acid secretion caused by gastrinomas. Somatostatin analogs can inhibit the secretion of multiple hormones and these are now the cornerstone for treating patients with a gastroenteropancreatic NET. However, peptide receptor radionuclide therapy (PRRT) with radiolabeled somatostatin analogs and everolimus can also decrease symptoms of hypersecretion and increase progression-free survival. Several factors affect the survival in patients with a functioning NET. Complications of hypersecretion negatively impact survival; however, secretion of hormones is also often a sign of a well-differentiated NET and due to the symptoms, functioning NETs can be detected in an earlier stage suggesting a positive effect on prognosis. The effect on survival is also dependent on the type of hormone being secreted. This review aims to study the effect of hormone secretion on the prognosis of NETs with the contemporary treatments options available today.
神经内分泌肿瘤（NETs）引起的激素分泌过多综合征的治疗可能是一项重大挑战。起源于小肠的NETs常分泌5-羟色胺，导致潮红、腹泻和瓣膜纤维化，严重时可导致脱水或心力衰竭。胰腺来源的NETs可分泌多种激素，如胰岛素、胰高血糖素和胃泌素，导致不同的临床综合征。历史上，功能性NETs患者的死亡率较高，原因是激素分泌过多引起的并发症。现在有几种药物降低了死亡率：质子泵抑制剂可减少胃泌素瘤引起的胃酸分泌。生长抑素类似物可抑制多种激素的分泌，目前是治疗胃肠胰NETs患者的基石。然而，用放射性标记的生长抑素类似物进行肽受体放射性核素治疗（PRRT）和依维莫司也可减轻分泌过多症状并延长无进展生存期。有几个因素影响功能性NETs患者的生存。分泌过多的并发症对生存有负面影响；然而，激素分泌通常也是高分化NET的一个标志，并且由于症状，功能性NETs可在早期被检测到，提示对预后有积极影响。对生存的影响还取决于所分泌激素的类型。本综述旨在研究激素分泌对当今可用的当代治疗方案下NETs预后的影响。
