The condition commonly presents with flushing and diarrhoea; other clinical features include wheeze, palpitations, telangiectasia, and abdominal pain. Classic facies and cardiac signs are features of examination. Diagnosis is confirmed by elevated levels of urinary 5-hydroxyindoleacetic acid (5-HIAA); chromogranin A is often elevated in the presence of liver metastases.

BMJ Best Practice is an evidence-based point of care tool for healthcare practitioners.

To continue reading and access all of BMJ Best Practice's pages you'll need to log in or start a free trial.

You can access through your institution if your hospital, university, trust or other institution provides access to BMJ Best Practice through either OpenAthens or Shibboleth.

Use of this content is subject to our disclaimer