Carcinoid syndrome (CS) is a debilitating disease that affects approximately 20% of patients with neuroendocrine neoplasms (NEN). Due to the increasing incidence and improved overall survival of patients with NEN over recent decades, patients are increasingly suffering from chronic and refractory CS symptoms. At present, symptom control is hampered by an incomplete understanding of the pathophysiology of this syndrome. This systematic review is the first to critically appraise the available evidence for the various hormonal mediators considered to play a causative role in CS. Overall, evidence for the putative mediators of CS was scarce and often of poor quality. Based on the available literature, data are only sufficient to agree on the role of serotonin as a mediator of CS-associated diarrhea and fibrosis. A direct role for tachykinins and an indirect role of catecholamines in the pathogenesis of CS is suggested by several studies. Currently, there is insufficient evidence to link histamine, bradykinin, kallikrein, prostaglandins, or motilin to CS. To summarize, available literature only sufficiently appoints serotonin and suggests a role for tachykinins and catecholamines as mediators of CS, with insufficient evidence for other putative mediators. Descriptions of CS should be revised to focus on these proven hormonal associations to be more accurate, and further research is needed into other potential mediators.

中文翻译：

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什么是类癌综合征？对其提议的调解人的批判性评估。


类癌综合征 (CS) 是一种使人衰弱的疾病，影响约 20% 的神经内分泌肿瘤 (NEN) 患者。近几十年来，由于 NEN 患者发病率的增加和总生存率的提高，越来越多的患者患有慢性和难治性 CS 症状。目前，对该综合征病理生理学的不完全了解阻碍了症状控制。本系统综述首次对被认为在 CS 中起因果作用的各种激素介质的现有证据进行了严格评估。总体而言，假定的 CS 调节因素的证据很少，而且质量往往很差。根据现有文献，数据仅足以就血清素作为 CS 相关腹泻和纤维化介质的作用达成一致。多项研究表明速激肽在 CS 发病机制中的直接作用和儿茶酚胺的间接作用。目前，没有足够的证据表明组胺、缓激肽、激肽释放酶、前列腺素或胃动素与 CS 相关。总而言之，现有文献仅充分指定了血清素，并暗示了速激肽和儿茶酚胺作为 CS 介质的作用，而对于其他假定介质的证据不足。应修改 CS 的描述，以更加准确地关注这些已证实的激素关联，并且需要对其他潜在介质进行进一步研究。