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Carcinoid syndrome

Incidence: 1 per 500 000 per year.

Features

  1. intermittent cyanotic flushing
  2. diarrhoea & vomiting, abdominal cramps
  3. bronchoconstriction: cough, wheeze, dyspneoa
  4. pulmonary stenosis, tricuspid regurgitation (smooth muscle plaques deposited in endocardium).
  5. hepatomegaly (metastases)

May also present with features of ↑gastrin, ↑ACTH, ↑insulin.

Δx. excess 5HIAA in 24h urine.

Causes

Neuroendocrine tumours of
  1. appendix
  2. rectum
  3. lungs (usually central)
  4. ileum, stomach, colon
  5. pancreas
  6. biliary tree
  7. liver
Midgut tumours 50%. Bronchial tumours 10%. Gut carcinoids are silent unless they metastasise to the liver.

Treatment

  1. Surgical resection
  2. Symptomatic relief Rx. octreotide
  3. Tumour targetted radiotherapy with 131I-MIBG