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Practice Easily Missed?

Carcinoid syndrome

BMJ 2010; 341 doi: (Published 23 August 2010) Cite this as: BMJ 2010;341:c3941
  1. R Srirajaskanthan, specialist registrar1,
  2. D Shanmugabavan, general practitioner 2,
  3. J K Ramage, consultant gastroenterologist 1
  1. 1Institute of Liver Studies, King’s College Hospital, London SE5 9RS
  2. 2Bexley Care Trust, Bexley Heath DA7 6HZ
  1. Correspondence to: R Srirajaskanthan r.srirajaskanthan{at}
  • Accepted 23 June 2010

Carcinoid syndrome consists of symptoms of facial flushing, diarrhoea, and episodic abdominal pain.1 It is caused by kinins and serotonin secreted from neuroendocrine tumours that arise from neuroendocrine cells found in almost all organs of the body, most commonly the gastrointestinal tract. Only 30-40% of neuroendocrine tumours cause clinical syndromes; ileal neuroendocrine tumours most commonly cause carcinoid syndrome. Over 95% of patients with carcinoid syndrome have metastatic disease. Hormones secreted by the primary tumour are metabolised by the liver and so do not cause symptoms, but symptoms may develop with liver metastases, as these release hormones that bypass the enterohepatic circulation and enter the systemic circulation.

Case scenario

A 40 year old man presented with a three year history of diarrhoea and episodic abdominal pain with no clear precipitating factors. After coeliac disease was excluded a provisional diagnosis of irritable bowel syndrome was made and various treatments were tried, without benefit. Over the past year his wife and children had noticed intermittent facial flushing. Ultrasound of the abdomen, performed by his general practitioner for his persistent abdominal cramping, identified liver lesions. On referral to a gastroenterologist, 5-hydroxyindoleacetic acid (5HIAA) in urine was found to be raised, consistent with findings of carcinoid syndrome. Liver biopsy of the liver lesions confirmed a neuroendocrine tumour.

How common is it?

  • The incidence of all neuroendocrine tumours is 3-5 cases per 100 000 population, according to the US surveillance epidemiology and end results database.2 These tumours are slow growing, and their prevalence is much higher, 35 per 100 000 cases3 …

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