Abstract
The background to this study was that factors associated with carcinoid heart disease (CHD) and its impacts on overall survival (OS) are scantly investigated in patients (pts) with neuroendocrine tumors (NETs). In terms of materials and methods, a retrospective multicenter cohort study was conducted of factors associated with CHD in advanced NET pts with carcinoid syndrome (CS) and/or elevated urinary 5-hidroxyindole acetic acid (u5HIAA). CHD was defined as at least moderate right valve alterations. The results were the following: Among the 139 subjects included, the majority had a midgut NET (54.2%), 81.3% had CS, and 93% received somatostatin analogues. In a median follow-up of 39 months, 48 (34.5%) pts developed CHD, with a higher frequency in pts treated in public (77.2%) versus private settings (22.9%). In a multivariate logistic regression, unknown primary or colorectal NETs (Odds Ratio (OR) 4.35; p = 0.002), at least 50% liver involvement (OR 3.45; p = 0.005), and being treated in public settings (OR 4.76; p = 0.001) were associated with CHD. In a Cox multivariate regression, bone metastases (Hazard Ratio {HR} 2.8; p = 0.031), CHD (HR 2.63; p = 0.038), and a resection of the primary tumor (HR 0.33; p = 0.026) influenced the risk of death. The conclusions were the following: The incidence of CHD was higher in pts with a high hepatic tumor burden and in those treated in a public system. Delayed diagnosis and limited access to effective therapies negatively affected the lives of NET patients.
Highlights
20–30% of patients with neuroendocrine tumors (NETs) are diagnosed with carcinoid syndrome (CS) in the United States [1], and it is usually associated with liver metastases and reduced overall survival [2,3]
In this multicenter retrospective cohort study, the largest conducted in Latin America and among the largest series of carcinoid heart disease (CHD) cases worldwide, we evaluated the incidence of CHD and the impact on overall survival (OS) of patients with advanced NETs, in addition to the already known adverse prognostic factors
Factors independently associated with CHD were treatment delivered in a public setting, unknown primary or colorectal NET, and at least 50% liver involvement by metastases
Summary
20–30% of patients with neuroendocrine tumors (NETs) are diagnosed with carcinoid syndrome (CS) in the United States [1], and it is usually associated with liver metastases and reduced overall survival [2,3]. Carcinoid syndrome, characterized by flushing, abdominal cramps, diarrhea, and bronchospasm [1,4,5], is caused by the secretion of vasoactive substances such as serotonin, histamine, prostaglandins, and tachykinins [1,5,6,7,8]. The secretion of these substances, in particular serotonin, can induce tissue fibrosis and lead to complications such as mesenteric, peritoneal, and endocardial fibrosis [8,9]. Because many patients with CHD do not present with symptoms until cardiopathy is in advanced stages [6,7], international guidelines recommend screening for CHD with an echocardiogram in patients with elevated urinary 5-hidroxyindole acetic acid (u5HIAA) (a metabolite of serotonin) independently of carcinoid syndrome symptoms [9,12]
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