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类癌综合征的诊断与治疗进展。

Recent Advances in the Diagnosis and Management of Carcinoid Syndrome.

机构信息

Ramaiah Medical College, MSR Nagar, Mathikere, Bengaluru, Karnataka 560054, India.

Ramaiah Medical college, MSR Nagar, Mathikere, Bengaluru, Karnataka 560054, India.

出版信息

Dis Mon. 2022 Jul;68(7):101304. doi: 10.1016/j.disamonth.2021.101304. Epub 2021 Dec 28.

Abstract

Carcinoid syndrome, a paraneoplastic condition linked with the release of multiple humoral factors, affects around 30-40% of patients with well-differentiated neuroendocrine tumours. Carcinoid syndrome has a major and unfavourable impact on patients' quality of life; it raises costs when compared to non-functioning neuroendocrine tumours; and it causes patients' lifestyles to alter, such as food, job, physical activity, and social life. Somatostatin analogues have been the first-line therapy for individuals with neuroendocrine tumours and carcinoid disease for decades. While these drugs give considerable relief from carcinoid syndrome symptoms, clinical progression is unavoidable, necessitating further research into newer treatment measures. Carcinoid tumours are sometimes difficult to diagnose because of their vague or nonspecific symptoms. There have been several advancements in all aspects of carcinoid syndrome, as well as novel therapeutics, in the previous few years. New epidemiological studies show that it is becoming more common; increasing insights into the pathogenesis of its various clinical manifestations and its natural history: definition of prognostic factors; new methods to verify its presence; the development of new drugs to treat its various manifestations, both initially and in somatostatin-refractory cases; and an increased understanding of the pathogenesis, natural history, and management of the disease. An all language literature search was conducted on MEDLINE, COCHRANE, EMBASE, and Google Scholar till November 2021. The following search strings and Medical Subject Headings (MeSH) terms were used: "Recent advances", "Carcinoid syndrome", "Neuroendocrine Neoplasms" and "Carcinoid heart disease". We comprehensively reviewed the literature on the pathogenesis, clinical features, and newer treatment modalities for Carcinoid Syndrome. Recent advancements in research and management have resulted from advances in our understanding of the aetiology of carcinoid syndrome. The development of molecular indicators of aggressiveness improved serum tumour markers, and the molecular aetiology of carcinoid heart disease are all possible because of advances in molecular biology. We conducted a comprehensive review to update knowledge regarding the pathophysiology, diagnostic protocols, and current and newer treatments for carcinoid syndrome, which presently requires a multidisciplinary approach, due to the complexity of the illness's aetiology, diagnosis, and therapy.

摘要

类癌综合征是一种与多种体液因子释放有关的副瘤综合征,影响大约 30-40%的分化良好的神经内分泌肿瘤患者。类癌综合征对患者的生活质量有重大且不利的影响;与无功能性神经内分泌肿瘤相比,它会增加成本;并导致患者的生活方式发生改变,如食物、工作、身体活动和社交生活。生长抑素类似物几十年来一直是神经内分泌肿瘤和类癌疾病患者的一线治疗药物。虽然这些药物可以显著缓解类癌综合征症状,但临床进展是不可避免的,因此需要进一步研究新的治疗措施。类癌肿瘤由于其模糊或非特异性症状,有时难以诊断。在过去的几年中,类癌综合征的各个方面以及新型治疗方法都取得了一些进展。新的流行病学研究表明,它变得越来越普遍;对其各种临床表现和自然史的发病机制有了更多的了解:定义预后因素;新的方法来验证其存在;开发新的药物来治疗其各种表现,包括初始和生长抑素难治性病例;以及对疾病的发病机制、自然史和管理有了更深入的了解。我们在 MEDLINE、COCHRANE、EMBASE 和 Google Scholar 上进行了全面的语言文献检索,检索时间截至 2021 年 11 月。我们使用了以下搜索字符串和医学主题词 (MeSH) 术语:“最新进展”、“类癌综合征”、“神经内分泌肿瘤”和“类癌心脏病”。我们全面回顾了关于类癌综合征发病机制、临床特征和更新治疗方法的文献。对类癌综合征发病机制的深入了解促进了研究和管理方面的最新进展。由于分子生物学的进步,侵袭性的分子标志物的发展提高了血清肿瘤标志物,以及类癌心脏病的分子病因学,这一切都是可能的。我们进行了全面的综述,以更新关于类癌综合征的病理生理学、诊断方案以及当前和更新治疗方法的知识,由于该疾病的病因、诊断和治疗的复杂性,目前需要多学科方法。

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