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What is multiple endocrine neoplasia?

The multiple endocrine neoplasia (MEN) syndromes are a group of genetically inherited diseases that result in cancer or dysfunction in multiple organs. Cancer may arise in multiple organs at the same time, or a person may develop one cancer, then a second cancer in another organ, many years later. These cancers also often arise in younger people and may be more aggressive than ‘sporadic’ cancers – those that do not have a genetic inheritance pattern.

These syndromes can be divided into two groups, MEN-1 and MEN-2

MEN-1

This syndrome affects about 2 - 20 in every 100,000 people and is caused by a heritable defect in the MEN-1 tumour suppressor gene. If one of your parents has this syndrome, you have a 50 per cent chance of having the syndrome yourself. The syndrome most commonly affects the parathyroid gland (four small glands on the front of your thyroid), pancreas and pituitary gland (a small gland at the base of your brain). However, a patient with MEN-1 may also develop cancer of the small intestines, adrenal glands and lipomas (fatty lumps on your skin).

MEN-2

MEN-2 can be further subdivided into type 2A and type 2B. In type 2A people develop medullary thyroid cancer (MTC), phaeochromocytoma and an over-active parathyroid gland. Type 2B is characterised by MTC, phaeochromocytoma and cancer on the lips or tongue. In this syndrome there is a genetic mutation in the 'RET proto-oncogene' (see RET gene mutation testing in Tests).


Last Review Date: March 15, 2015