Abstract
Carcinoid syndrome is the constellation of symptoms mediated by humoral factors produced by some carcinoid tumors. It consists primarily of vasomotor symptoms, gastrointestinal hypermotility, hypotension, and bronchospasm, due to the production and release of vasoactive substances. Carcinoid heart disease occurs in more than 50% of patients with carcinoid syndrome; in some cases, it represents the initial manifestation of the disease. We report the case of a 75-year-old woman with a metastatic neuroendocrine tumor admitted to the emergency room for fatigue and heart failure. Transthoracic echocardiography showed severe tricuspid and pulmonic regurgitation suggesting carcinoid heart disease. A hypervascular retroperitoneal mass was found on abdominal computed tomography, which seemed to arise from the mesenteric artery, anteriorly to the abdominal aorta. Unfortunately, our patient was neither a candidate for mass resection nor for cardiac surgery due to advanced metastatic disease and poor clinical condition. Additionally, we performed a systematic literature review of carcinoid heart disease focusing on typical echocardiographic findings.
Highlights
Carcinoid syndrome (CS) is a clinical characteristic of welldifferentiated neuroendocrine tumors (NETs), seen in those arising from the midgut, and less commonly from the lung and the pancreas.[1]
The most common manifestations of CS are vasomotor symptoms, hypotension, secretory diarrhea, abdominal cramps, and bronchospasm. This syndrome can lead to clinically severe complications caused by tissue fibrotic degeneration, such as carcinoid heart disease (CHD), mesenteric and retroperitoneal fibrosis, and debilitating diarrhea.[2]
Abdominal ultrasound was nondiagnostic due to intestinal bloating and visceral obesity; computed tomography (CT) of the abdomen was performed (Figure 1), showing a hypervascular retroperitoneal mass (8.5 × 11 × 6.5 cm), which seemed to arise from the mesenteric artery, anteriorly to the abdominal aorta
Summary
Carcinoid syndrome (CS) is a clinical characteristic of welldifferentiated neuroendocrine tumors (NETs), seen in those arising from the midgut (ie, small intestine, appendix, and proximal colon), and less commonly from the lung and the pancreas.[1]. The most common manifestations of CS are vasomotor symptoms (flushing), hypotension, secretory diarrhea, abdominal cramps, and bronchospasm This syndrome can lead to clinically severe complications caused by tissue fibrotic degeneration, such as carcinoid heart disease (CHD), mesenteric and retroperitoneal fibrosis, and debilitating diarrhea.[2]. Blood pressure was 164/85 mm Hg, heart rate was 78 bpm (sinus rhythm), peripheral capillary oxygen saturation was 90% with normal respiratory rate, and body mass index was 32 kg/m2. The left-sided valves were normal in appearance and function These findings, along with extremely elevated serum levels of chromogranin A (>4016 ng/mL, normal value = 20-185 ng/mL), raised the suspicion of an NET originating in the small intestine with secondary spread to the mesentery, liver, and lymph nodes, with associated CS with heart involvement. Medical treatment with somatostatin analogues was started to reduce hormone-related symptoms, but the patient died after a few months of medical therapy
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