Carcinoid - NYSORA

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Learning objectives

  • Describe carcinoid and carcinoid syndrome
  • Recognize the symptoms and signs of carcinoid and carcinoid syndrome
  • Anesthetic management of a patient with carcinoid

Definition and mechanisms

  • A carcinoid is a slow-growing neuroendocrine tumor derived from enterochromaffin or Kulchitsky cells
  • Carcinoid tumors usually begin in the gastrointestinal tract (i.e., stomach, appendix, small intestine, colon, or rectum), or in the lungs
  • Carcinoid syndrome (± 20% of patients with carcinoid tumors) results from the direct release of vasoactive amines (e.g., serotonin and histamine) and peptides into the systemic circulation, usually from hepatic metastases associated with midgut (jejunum, ileum, appendix, and cecum) carcinoids

Signs and symptoms

Some tumors do not cause any signs or symptoms. When they do occur, signs and symptoms are usually vague and depend on the tumor’s location.

LocationSigns and symptoms
Gastrointestinal tractAbdominal pain
Nausea, vomiting, and bowel obstruction
Rectal bleeding
Rectal pain
Skin flushing
LungsChest pain
Skin flushing
Weight gain
Pink or purple marks on the skin (looking like stretch marks)

Clinical manifestations of carcinoid syndrome

Sign/symptomFrequencyCharacteristicsInvolved mediators
Flushing85-90%Foregut: long-lasting purple face and neck
Midgut: short-lasting, pink/red
Severe flushing associated with hypotension and tachycardia
Kallikrein, 5-hydroxytryptophan(5-HTP; chemical precursor and metabolic intermediate in the biosynthesis of serotonin), histamine, substance P, prostaglandins (PGs)
GI hypermotility70-80%Secretory diarrhea, nausea, vomitingGastrin, 5-HTP, histamine, PGs, vasoactive intestinal peptides (VIPs)
Abdominal pain35%ProgressiveSmall bowel obstruction, hepatomegaly, ischemia
Right-sided heart failure30%Dyspnea5-HTP, substance P
Left-sided heart failure10%Dyspnea5-HTP, substance P
Bronchospasm15%WheezingHistamine, 5-HTP
Pellagra5%Dermatitis, diarrhea, dementiaNiacin deficiency

Risk factors


The cells of carcinoid tumors can secrete hormones and other chemicals, causing a range of complications including:

  • Carcinoid syndrome: Causes skin flushing, chronic diarrhea, and difficulty breathing (i.e., bronchospasm), among other signs and symptoms (see table above)
  • Carcinoid heart disease: Carcinoid tumors may secrete hormones causing thickening of the endocardium of the cardiac chambers, valves, and blood vessels, leading to leaky heart valves and heart failure (i.e., typically right-sided heart failure)
  • Other endocrine disorders: Carcinoid tumors can also secrete growth hormone and adrenocorticotropin-releasing hormone, respectively leading to acromegaly and Cushing’s syndrome


  • Surgery: When detected early, possible to dissect completely
  • Medications: To block the excess hormone secretion by the tumor and reduce the signs and symptoms, and slow tumor growth
  • Chemotherapy: Recommended for advanced tumors that cannot be removed surgically
  • Targeted drug therapy: Usually combined with chemotherapy for advanced tumors
  • Treatment for tumors with metastases to the liver: Surgery to remove part of the liver, hepatic artery embolization (blocking blood flow to the liver), and using heat (i.e., radiofrequency ablation) and/or cold (i.e., cryoablation) to kill cancer cells


carcinoid, carcinoid crisis, carcinoid syndrome, octreotide, antihistamines, diphenhydramine, loratadine, ranitidine, famotidine, benzodiazepines, anxiolytics, midazolam, diazepam, crystalloids, colloids, vasoconstrictor, phenylephrine, metaraminol, noradrenaline

Keep in mind

  • The goal is to prevent, recognize, and treat perioperative carcinoid crises
  • Triggers include histamine-releasing drugs, vasoactive drugs, succinylcholine; tumor manipulation; and hypovolemia, hypoxia, hypothermia, hypercarbia
  • Treatment of perioperative bronchospasms include octreotide, steroids, histamine blockade (diphenhydramine), and Atrovent
  • Avoid beta-agonists, theophylline, and epinephrine to treat perioperative bronchospasms

Suggested reading

  • Kaltsas G, Caplin M, Davies P, et al. ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Pre- and Perioperative Therapy in Patients with Neuroendocrine Tumors. Neuroendocrinology. 2017;105(3):245-254.
  • Powell B, Al Mukhtar A, Mills GH. Carcinoid: the disease and its implications for anaesthesia. Continuing Education in Anaesthesia Critical Care & Pain. 2011;11(1):9-13.
  • Mancuso K, Kaye AD, Boudreaux JP, et al. Carcinoid syndrome and perioperative anesthetic considerations. J Clin Anesth. 2011;23(4):329-341.

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