ABSTRACT
Multiple endocrine neoplasia (MEN) syndromes are a group of endocrine disturbances that affect hormone-secreting glands. These are rare autosomal dominant conditions that predispose affected individuals to benign and malignant tumours of the pituitary, thyroid, parathyroids, adrenals, endocrine pancreas, paraganglia or non-endocrine organs. The classic MEN syndromes include MEN type 1 (MEN1) and MEN type 2 (MEN2). MEN1 is an autosomal dominant disorder with a prevalence of around 2 per 100 000. It is characterised by the combined occurrence of tumours of the parathyroid glands, the pancreatic islet cells and the anterior pituitary. MEN2 is an autosomal dominant disorder with an estimated prevalence of 2.5 per 100 000 in the general population. Both sexes are nearly equally affected. MEN2 syndrome is often first suspected when a patient is found to have one or more of the tumours described in the syndrome, usually medullary thyroid cancer, or a family history.
