Neuroendocrine tumours (NET) arise from the malignant growth of neuroendocrine cells, a system of paracrine cells distributed throughout the body. Most NETs arise from the gastrointestinal tract, most commonly in the small bowel and the lungs. About half of NETs occur in the small bowel and about one quarter in the lungs. Other forms of NETs are widespread and less common; the pancreatic NETs (PNET) account for about one-tenth of the prevalence of total NETs.

Neuroendocrine cells and NETs produce paracrine and vasoactive compounds and proteins through degranulation and this can give rise to syndromic presentations due to the secretion of these active compounds. This causes much of the morbidity associated with these tumours, including changes in GI tract function, fibrosis both peritumoural and cardiac, vascular changes including generalised flushing and many other less common symptoms.

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