Key result
Metastasized ileal NET can present with fatal fulminant systemic vasoconstriction, possibly driven by elevated catecholamines, leading to heart failure and severe circulatory insufficiency.
Case Report (n=1)
Highlights a rare case of fatal systemic vasoconstriction and subsequent heart failure in a patient with metastatic ileal NET, likely driven by catecholamine release.
Clinicians should consider rare NETs in unexplained vasoconstriction with HF; this case leaves open mechanistic confirmation and therapeutic implications.
An increased release of serotonin secreted by ileal NETs is thought to be the major factor causing the carcinoid syndrome. However, in acutely arising carcinoid crisis also other vasoactive factors may lead to hazardous fluctuations in blood pressure and bronchial constriction. In rare cases, systemic vasoconstriction can be observed, probably caused by catecholamines or similar acting substances. Here, we report a fatal case of fulminant systemic vasoconstriction possibly caused by catecholamines in a patient with metastasized ileal NET. The vasospasm was detected by CT-angiography, and hemodynamic monitoring revealed a high systemic vascular resistance. Epinephrine, norepinephrine, and chromogranin A levels in plasma were elevated as was the urinary 5-hydroxyindoleacetic acid (5-HIAA). The cause of death was heart failure due to severe circulatory insufficiency. The progression of the tumor disease was confirmed by autopsy.
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Stenzel et al. (2017) conducted a case report in Metastatic Small-Intestinal NET (n=1). Metastasized ileal NET can present with fatal fulminant systemic vasoconstriction, possibly driven by elevated catecholamines, leading to heart failure and severe circulatory insufficiency.
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