(Björk syndrome, argentaffinoma)

Rare. Clinical picture produced by the secretion of vasoactive substances produced by carcinoid tumors. Carcinoid tumors are neuroendocrine tumors that originate from the APUD (Amine Precursor Uptake Decarboxylation) cells; they are generally located in the digestive tract (75 %), sometimes in the lung, pancreas or thymus. These tumors are slowly growing and are not very symptomatic. They secrete vasoactive substances (serotonin, histamine, bradykinin) that cause clinical signs (carcinoid syndrome) when they are not metabolised at liver level i.e. in case of primitive location outside the area of drainage by the portal vein or in case of liver metastases. In children, the most common location is the appendix and the carcinoid tumor is a fortuitous discovery at the histologic examination. The carcinoid syndrome is defined as a crisis with hypotension or hypertension, skin flush, bronchoconstriction, diarrhea. These symptoms are caused by the release of vasoactive substances  in response to stress, exercise or ingestion of foods rich in serotonin (alcohol, bananas, cheese, coffee). Some patients may also show signs of pellagra following secondary tryptophan deficiency (from which serotonin is synthesized): dermatitis, diarrhea, signs of dementia.

 Carcinoid crisis : very severe form of carcinoid syndrome thatcan be caused by the manipulation of the tumor, its necrosis following chemotherapy or hepatic artery embolization/ligation. It can also occur during emotions, alcohol intake or at induction of anesthesia. 

Cardiac effects: the presence of elevated kinin blood levels can lead to progressive fibrosis of the endocardium and progressive valvular involvement. These cardiac lesions are generally located in the right cavities: tricuspid stenosis or insufficiency, pulmonary stenosis or  insufficiency and finally right heart failure. However, in case of bronchial tumor, pulmonary hypertension, or the involvement of the left cardiac cavities is possible.

Treatment: the treatment by octeotride allows to decrease symptoms and avoid carcinoid crisis. The chemoembolization of the hepatic metastases helps to improve survival.

Anesthetic implications: 

continue treatment with IV octeotride or somatostatin during perioperative period; cardiac ultrasound: valvular lesions; avoid succinylcholine (fasciculations?); avoid the agents stimulating the release of histamine; in presence of high blood levels of serotonin, there can be a delay in awakening; in case of hypotension and bronchospasm, be careful as the use of catecholamines may aggravate the release of vasoactive substances by the tumor: the administration of a bolus dose of octeotride could be more effective; in case of neuraxial block, careful titration of the dose and optimized volemia to prevent hypotensive episodes.

References : 

-         Thorson A, Bjork G, Bjorrkman G, Waldenstrom J. 
Malignant carcinoid of the small intestine with metastases to the liver, valvular disease of the right side of the heart, peripheral vasomotor symptoms, bronchoconstriction, and an unusual type of cyanosis: a clinical and pathological syndrome. 
Am Heart J 1954; 47: 795-817.

-        Spunt SL, Pratt CB, Rao BN, Pritchard M, Jenkins JJ, Hill DA, Cain AM, Pappo AS. 
Childhood carcinoid tumors: the St Jude Children’s Research Hospital experience. 
J Pediatr Surg 2000; 35:1282-6.

-         Mancuso K, Kaye AD, Boudreaux JP, Fox CJ, Kalarickal PL, Gomez S, Primeaux PJ.  
Carcinoid syndrome and perioperative anesthetic considerations.
J Clin Anesth 2011; 23: 329-41.

Updated: November 2019