Carcinoid Syndrome


Carcinoid syndrome is a term used to describe a group of signs and symptoms occurring following the release of chemical agents by carcinoid tumours. Carcinoid syndrome occurs only in about 2 – 10 % of patients with this tumour.

These small white to yellowish tumours originate from the cells of the intestinal lining. They are commonest in the wall of the appendix, and the small part of the intestine called ileum.

The chemicals produced by these tumours include bradykanins, serotonin, cathecholamines, 5HT-derivatives, histamine, substance P and prostaglandins.

Ordinarily, these chemical agents when produced by carcinoid tumours located within the gut, they are carried in the blood to liver where there are broken down. This is because all the blood coming from the gut passes through the liver first before getting to other parts of the body.

Since these chemicals are destroyed in the liver first before getting to the general blood stream, carcinoid tumours on the gut wall do not cause problems until they spread to the liver.

The carcinoid tumours do spread or metastasize to distant organs like the liver, lungs and heart.

Such tumours outside the gut are what causes carcinoid syndrome. When they release those chemicals, these are carried in the blood stream out of the reach of the liver in the first instance. They travel to sites such as the brain, heart, lungs and even back to the intestines, causing the constellation of symptoms described in carcinoid syndrome.

Consumption of alcohol, cheese, chocolate, spicy foods, hot drinks, sex, physical or psychiatric excitements have been known to trigger this syndrome in some individuals.

The signs and symptoms making up carcinoid syndrome include:

• Severe abdominal pain

• Diarrhoea

• High fever

• Flushing

• Wheezing

• Low blood pressure and

• Heart disease

• Pellegra

• Confusion

• Skin diseases

These are discussed in greater details below.

Only about 2 – 10 % of patients with carcinoid tumour have metastatic disease leading to the development of carcinoid syndrome. 86% of these have the tumour involving their liver.

Other sites from which potent chemical hormones can be released causing this syndrome include:

Tumour of the Liver

Carcinoid Tumour of the Lungs

Carcinoid syndrome could also occur from primary gut carcinoid tumour following handling during operations like during appendicitis. Fatal exacerbation of this syndrome has been described in such condition.

We shall now discuss about the signs and symptoms, investigations, treatment and prognosis of carcnoid syndrome below:

Symptoms of Carcinoid Syndrome




Carcinoid tumours


Bailey & Love’s Short Practice of Surgery. 23 rd Edition.

C. D. Knox, C. D. Anderson, L. W. Lamps, R. B. Adkins, and C. W. PinsonLong-Term Survival after Resection for Primary Hepatic Carcinoid TumorAnn. Surg. Oncol., December 1, 2003; 10(10): 1171 - 1175.

Lung Cancer: Principles and Practiceedited by Harvey I Pass, David P Carbone, John D Minna, Andrew T Turrisi, David H Johnson

General Thoracic Surgery By Thomas W Shields, Joseph Locicero, Ronald B Ponn, Valerie W Rusch

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