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.

Prevention and Management of Hormonal Crisis during Theragnosis with LU-DOTA-TATE in Neuroendocrine Tumors. A Systematic Review and Approach Proposal

“…Correcting dehydration, electrolyte disorders, and hypoproteinemia are especially important, as well as avoiding alcohol, spicy food, and tryptophan rich food. Physical exertion may also exacerbate the symptoms of CS, so it should be avoided in the days prior to the administration of Lu-DOTA-TATE [ 24 , 27 , 31 , 32 , 34 ]. In functional NETs, and specifically if they present CS, the first-line pharmacological treatment are SSAs.…”

“…Cyproheptadine, a serotonin receptor blocker, can be used in doses of 4 mg/8 h. Telotristat ethyl has been found to reduce serotonin production by inhibiting tryptophan hydroxylase (TPH). It is indicated in combination with SSA therapy in adults with CS diarrhea inadequately controlled by SSA therapy [ 17 , 24 , 29 , 32 ]. Premedication with benzodiazepines and antihistamines can be useful to reduce anxiety before treatment with PRRT, but its administration is controversial, since histamine release may occur, especially with gastric or bronchial carcinoids [ 29 ].…”

Carcinoid Crisis: A Misunderstood and Unrecognized Oncological Emergency

“…Analyzing the possible mechanism behind hormonal secretion during Lu-octreotate therapy (tumor lysis vs. discontinuation of short-acting somatostatin analog vs. emotional stress response to hospitalization vs. administration of arginine and lysine), the Authors concluded that hormonal crises should be managed with an infusion of somatostatin analogs i.v., fluids, corticosteroids and correction of electrolyte disturbances. More recently, Stenzel et al came to the same conclusions [45]. Moreover, the Australian group of Tapia Rico et al first proposed a protocol to prevent and manage severe CC and specifically defined which patients would benefit most ("high-risk patients") from premedication to PRRT therapy with corticosteroids and bolus dose of octreotide sc [46].…”

Corrigendum to “Fatal Systemic Vasoconstriction in a Case of Metastatic Small-Intestinal NET”

“…In the article titled “Fatal Systemic Vasoconstriction in a Case of Metastatic Small-Intestinal NET” [ 1 ], the first and last names of all the authors were reversed. The revised authors' list is shown above and updated in place.…”