ENETS Center of Excellence, Section of Endocrinology, Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands.
Maimonides Institute for Biomedical Research of Cordoba (IMIBIC), Córdoba, Spain.
Endocr Relat Cancer. 2019 Mar;26(3):R145-R156. doi: 10.1530/ERC-18-0495.
Carcinoid syndrome (CS) is a debilitating disease caused by functional neuroendocrine tumors. Several treatment options are available to alleviate the hormonal symptoms, but their relative efficacy is unknown. Online databases were searched for publications on the treatment of CS symptoms. Independent reviewers assessed relevant publications for study quality and outcome. Meta-analysis of the outcomes of the intervention on CS-related symptoms was stratified by the type of treatment. We found 3682 therapeutic interventions on CS-specific outcomes were collected from 93 studies. Overall, the study qualities were poor with only six randomized controlled clinical trials. The somatostatin analogs octreotide and lanreotide induced symptomatic improvement in 65-72% and biochemical response in 45-46% of patients. An increase in dose or frequency or interclass switch led to a reduction of flushes and/or diarrhea in 72-84% of cases. Retrospective, institutional series showed that liver-directed therapy can improve symptoms in 82% of CS patients with a liver-dominant disease. The serotonin synthesis inhibitor telotristat ethyl reduced bowel movements in 40% of patients with diarrhea refractory to somatostatin analogs. Interferon-alpha controlled CS symptoms in 45-63% of cases. Favorable response has been noted after radionuclide therapy in subgroup analyses of studies not specifically involving CS patients. Chemotherapy and everolimus did not induce a significant response in the CS. We conclude that several treatment lines can be offered to patients suffering from the carcinoid syndrome. Initiation of randomized controlled trials with a primary outcome on carcinoid syndrome symptoms is strongly recommended.
类癌综合征（CS）是一种由功能性神经内分泌肿瘤引起的使人虚弱的疾病。有几种治疗方法可用于缓解激素症状，但它们的相对疗效尚不清楚。在线数据库中搜索了关于 CS 症状治疗的出版物。独立评审员评估了相关出版物的研究质量和结果。根据治疗类型对 CS 相关症状干预的结果进行了荟萃分析。我们从 93 项研究中收集了 3682 项针对 CS 特定结局的治疗干预措施。总体而言，研究质量较差，仅有 6 项随机对照临床试验。生长抑素类似物奥曲肽和兰瑞肽可使 65-72%的患者出现症状改善，45-46%的患者出现生化缓解。增加剂量或频率或类间转换可使 72-84%的病例潮红和/或腹泻减少。回顾性机构系列表明，肝定向治疗可使 82%肝脏为主疾病的 CS 患者的症状得到改善。血清素合成抑制剂托瑞司他乙酯可使 40%对生长抑素类似物治疗无效的腹泻患者减少排便次数。干扰素-α可控制 45-63%的 CS 症状。亚组分析显示，放射性核素治疗在非专门针对 CS 患者的研究中可使 CS 得到缓解。化疗和依维莫司在 CS 中未引起显著反应。我们得出结论，可向患有类癌综合征的患者提供几种治疗方案。强烈建议启动以类癌综合征症状为主要结局的随机对照试验。
