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Pathology
Tumour of enterochromaffin cells of neural crest secreting 5-hydroxytriptamine
Location: Appendix (45%), ileum (30%), rectum (20%), ovary/testis (5%)
80% tumours >2cm metastasize.Aetiology
Commonest neuroendocrine tumour.
Peak age >30 years
Signs
Flushing, right-sided heart valve disease, peripheral oedema
Symptoms
Abdominal pain, diarrhoea, wheeze
Investigations
Urine: 24 hour collection for 5-hyroxyindoleacetic acid
Imaging: CT/MRI to locate tumour
Treatment
Medical: Ocreotide (blocks tumour mediators and reduces peripheral effects)
and 5-HT antagonists
Surgical: Resection of tumour, chemotherapy
Prognosis
5 year survival – 95% for local disease
Heart failure, liver metastasis
Key Facts
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Key Images
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Key References
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