Fatal Systemic Vasoconstriction in a Case of Metastatic Small-Intestinal NET

Abstract

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.

Figure 1

(a) Skin with regional vasospasm; (b) opened heart, showing multiple small white metastases of a midgut NET.

Figure 2

Abdominal CT-scan. (a) Centralized perfusion with constricted peripheral intestinal arteries. (b) Reduced diameters of abdominal arteries. (A) Truncus coeliacus: 3 mm. (B) A. mesenterica sup.: 3 mm; aorta 15 mm.

Figure 3

Somatostatin receptor imaging with CT-scan (PET-CT) (a) showing extensive liver metastasis in the right liver lobe in 12/2011 and (b) additionally showing hydronephrosis.

Figure 4

3D reconstruction of the abdominal arteries showing the rarefication of the peripheral hepatic arteries. (A) Truncus coeliacus. (B) A. mesenterica sup. (C) Branch of the constricted hepatic artery.