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Ghrelin is a 28 amino-acid hormone with multiple functions. It is predominantly produced by the stomach but has also been detected in other organs, including the small intestine, pancreas, hypothalamus and pituitary, as well as in the immune system and almost every other normal human tissue examined. It is also present in neuroendocrine tumours, pituitary adenomas, endocrine tumours of the pancreas, breast tumours, and thyroid and medullary thyroid carcinomas. Ghrelin is a brain-gut peptide with growth hormone-releasing and appetite-inducing activities, and is the endogenous ligand of the G protein-coupled growth hormone secretagogue receptor (GHS-R). In this review we comprehensively summarize the available data regarding (a) the expression of ghrelin and the GHS-R in normal endocrine tissues and in pituitary adenomas and neuroendocrine tumours, (b) the levels of circulating ghrelin in patients with pituitary adenomas and neuroendocrine tumours and (c) the effects of ghrelin administration in these patients on the levels of other hormones and on the rate of proliferation of the tumour. It is clear that ghrelin has many more functions and is involved in many more processes than was initially postulated, and its endocrine, paracrine and autocrine effects play a role in its physiological and pathophysiological functions.