George Joel, Ramage John, White Benjamin, Srirajaskanthan Rajaventhan
Hampshire Hospitals NHS Trust, Basingstoke, United Kingdom of Great Britain and Northern Ireland.
Kings Health Partners NET Centre of Excellence, London, United Kingdom of Great Britain and Northern Ireland.
Endocr Oncol. 2023 Apr 20;3(1):e220077. doi: 10.1530/EO-22-0077. eCollection 2023 Jan 1.
Carcinoid syndrome is the most frequent hormonal complication associated with neuroendocrine neoplasms. It was first reported in 1954, and the classical symptoms are diarrhoea, flushing and abdominal pain. It is caused by the secretion of several vasoactive substances, the most prominent being serotonin, which play a pathophysiological role in the clinical symptoms which characterise carcinoid syndrome. Therefore, the focus of carcinoid syndrome treatment is to reduce serotonin production and hence improve the patient's quality of life. There are a variety of management options for carcinoid syndrome including medical, surgical and loco-regional interventional radiological procedures. The most widely used are somatostatin analogues with three clinically approved drugs: lanreotide and octreotide (first-generation) and pasireotide (second-generation). Both everolimus and interferon used in combination with octreotide have shown significant reduction in urinary 5-hydroxyindoleacetic acid compared to octreotide alone. Telotristat ethyl has been increasingly utilised for patients with symptoms despite taking somatostatin analogues. It has also been shown to have a significant improvement in bowel movement frequency which was associated with a significant improvement in quality of life. Peptide receptor radionuclide therapy has proven symptomatic improvement in patients with uncontrolled symptoms. Chemotherapy is primarily reserved for patients with high proliferation tumours, with limited research on the efficacy in reducing symptoms. Surgical resection remains the optimal treatment due to being the only one that can achieve a cure. Liver-directed therapies are considered in patients where curative resection is not possible. There are therefore numerous different therapies. This paper describes the pathophysiology and therapy of carcinoid syndrome.
类癌综合征是与神经内分泌肿瘤相关的最常见的激素并发症。它于1954年首次被报道，典型症状为腹泻、潮红和腹痛。它是由几种血管活性物质的分泌引起的，其中最主要的是血清素，这些物质在类癌综合征的临床症状中发挥病理生理作用。因此，类癌综合征治疗的重点是减少血清素的产生，从而提高患者的生活质量。类癌综合征有多种管理选择，包括药物、手术和局部区域介入放射学程序。使用最广泛的是生长抑素类似物，有三种临床批准的药物：兰瑞肽和奥曲肽（第一代）以及帕西瑞肽（第二代）。与单独使用奥曲肽相比，依维莫司和干扰素与奥曲肽联合使用均显示尿5-羟吲哚乙酸显著降低。尽管服用了生长抑素类似物，但对于有症状的患者，乙基替洛曲肽的使用越来越多。它还被证明能显著改善排便频率，这与生活质量的显著改善相关。肽受体放射性核素治疗已被证明能改善症状控制不佳患者的症状。化疗主要用于高增殖性肿瘤患者，关于其减轻症状疗效的研究有限。手术切除仍然是最佳治疗方法，因为它是唯一能实现治愈的方法。对于无法进行根治性切除的患者，考虑采用肝脏靶向治疗。因此有许多不同的治疗方法。本文描述了类癌综合征的病理生理学和治疗方法。
