Perioperative Management of Patients with Carcinoid Syndrome/Neuroendocrine Neoplasm

Background: Carcinoid tumours are neuroendocrine tumours that originate in the digestive tract. Carcinoid syndrome refers to a constellation of symptoms such as flushing, diarrhoea and bronchospasm that are mediated by elevated serum serotonin or its metabolites. Carcinoid crisis is a life-threatening complication of carcinoid syndrome. This review article provides an overview of clinical aspects relevant to the peroperative management of patients with carcinoid syndrome. It provides the relevant pathophysiological mechanisms. Special attention is paid to prevention and management of perioperative complications. Methods: This review article is based on a selective literature search in PubMed (Medline). Special attention is paid to current international guidelines and classification criteria. Results: Carcinoid syndrome is characterised by cutaneous flushing, bronchospasm and diarrhoea. Patients with carcinoid syndrome require careful preoperative evaluation and interdisciplinary preparation to avoid perioperative complications. Carcinoid heart disease is characterised by fibrotic plaques resulting in tricuspid regurgitation and pulmonary stenosis. Carcinoid crisis may be provoked by stress, anxiety, induction of anaesthesia, surgery, hypotension and hypothermia. Intravenous administration of somatostatin analogues can reverse intraoperative carcinoid crisis. Drugs that stimulate catecholamine or histamine release should be used with caution, as they may worsen hypotension. Conclusion: Endocrine disturbances and carcinoid crises increase the risk of surgery. Specific preparation and interdisciplinary collaboration are required to prevent potentially life-threatening circulatory complications. This review article provides recommendations for the perioperative management of carcinoid syndrome. Special attention is paid to pathophysiological and anaesthesiological aspects in the perioperative setting.