Carcinoid heart disease in patients with advanced small-intestinal neuroendocrine tumors and carcinoid syndrome: a retrospective experience from two European referral centers

IF 7.1 2区医学 Q1 ONCOLOGY

ESMO Open Pub Date : 2024-10-22 DOI:10.1016/j.esmoop.2024.103959

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引用次数: 0

Abstract

Background

Up to 50% of patients with advanced small-intestinal neuroendocrine tumors (SI-NETs) and carcinoid syndrome (CS) develop carcinoid heart disease (CHD). However, the true frequency and prognostic markers for CHD in CS are lacking. We described the real-world management of patients in two NET referral centers in this clinical context and relationships between clinical features, including CHD and overall survival (OS).

Patients and methods

This is a retrospective analysis of patients with stage IV SI-NET and CS, treated at the European Institute of Oncology in Milan and Uppsala University in Sweden between 2015 and 2021. CHD was defined as at least one moderate right-sided heart valve defect. Median OS and cumulative incidence of CHD were estimated from the diagnosis of metastatic disease, and the association between clinical parameters with both OS and occurrence of CHD was evaluated.

Results

We included 165 patients, with 97% having low-intermediate-grade SI-NETs and 86% having synchronous liver metastases. Ninety-eight patients (59%) became refractory to full label dose of somatostatin analogues and 25% developed a CHD. At CHD diagnosis, baseline urine 5-hydroxyindoleacetic acid (24-h u5-HIAA) value and plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) value were known in 76% of patients. Moderate-to-severe tricuspid insufficiency was the most common alteration of CHD. Prognosis was significantly impaired by CHD (multivariable hazard ratio for OS = 2.85, P < 0.001). The median OS from the CHD diagnosis was 4.5 years [95% confidence interval (CI) 2.1-7.2 years], and the 5-year survival rate was 34% (95% CI 13% to 57%).

Conclusions

In our study population of SI-NET patients with CS, more than half had a refractory carcinoid syndrome (RCS) and one-quarter developed a CHD, with a negative impact on OS. Therefore, it is recommended to screen and monitor patients with CS for CHD, ideally with a combination of u5-HIAA, NT-proBNP values, and echocardiography at CS baseline, preferably in NET referral centers.

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晚期小肠神经内分泌肿瘤和类癌综合征患者的类癌心脏病：两个欧洲转诊中心的回顾性经验。

背景：在晚期小肠神经内分泌肿瘤（SI-NETs）和类癌综合征（CS）患者中，高达50%的患者会出现类癌性心脏病（CHD）。然而，目前尚缺乏类癌性心脏病的真实发病率和预后指标。我们描述了两个NET转诊中心在这种临床背景下对患者的实际管理情况，以及包括CHD在内的临床特征与总生存期（OS）之间的关系：这是一项回顾性分析，对象是2015年至2021年间在米兰欧洲肿瘤研究所和瑞典乌普萨拉大学接受治疗的IV期SI-NET和CS患者。CHD定义为至少一个中度右侧心脏瓣膜缺损。从转移性疾病的诊断开始估算中位OS和CHD的累积发生率，并评估临床参数与OS和CHD发生率之间的关系：我们共纳入了165例患者，其中97%为中低分级SI-NET，86%为同步肝转移。98名患者（59%）对全标剂量的体生长抑素类似物产生了难治性，25%的患者出现了CHD。在诊断出心脏病时，76% 的患者已知尿液中 5-羟基吲哚乙酸（24 小时 u5-HIAA）的基线值和血浆中 N 端前脑钠肽 (NT-proBNP) 的值。中度至重度三尖瓣关闭不全是最常见的心脏病变。CHD会严重影响预后（OS的多变量危险比 = 2.85，P < 0.001）。自确诊CHD起的中位OS为4.5年[95%置信区间（CI）为2.1-7.2年]，5年生存率为34%（95% CI为13%-57%）：结论：在我们研究的SI-NET CS患者中，一半以上患有难治性类癌综合征（RCS），四分之一发展为慢性心肌梗死，对患者的OS有负面影响。因此，建议最好在NET转诊中心对CS患者进行CHD筛查和监测，最好在CS基线时结合u5-HIAA、NT-proBNP值和超声心动图检查。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

ESMO Open Medicine-Oncology

CiteScore

11.70

自引率

2.70%

发文量

255

审稿时长

10 weeks

期刊介绍： ESMO Open is the online-only, open access journal of the European Society for Medical Oncology (ESMO). It is a peer-reviewed publication dedicated to sharing high-quality medical research and educational materials from various fields of oncology. The journal specifically focuses on showcasing innovative clinical and translational cancer research. ESMO Open aims to publish a wide range of research articles covering all aspects of oncology, including experimental studies, translational research, diagnostic advancements, and therapeutic approaches. The content of the journal includes original research articles, insightful reviews, thought-provoking editorials, and correspondence. Moreover, the journal warmly welcomes the submission of phase I trials and meta-analyses. It also showcases reviews from significant ESMO conferences and meetings, as well as publishes important position statements on behalf of ESMO. Overall, ESMO Open offers a platform for scientists, clinicians, and researchers in the field of oncology to share their valuable insights and contribute to advancing the understanding and treatment of cancer. The journal serves as a source of up-to-date information and fosters collaboration within the oncology community.