Multiple endocrine neoplasia

Multiple endocrine neoplasia (MEN) is characterised by the occurrence of tumours involving 2 or more endocrine glands in a single patient.

MEN may be sporadic or inherited in an autosomal dominant pattern.

The subtypes of MEN are: 
  • MEN 1: 
    • Tumours of:
      • Parathyroid – 95% 
      • Pancreas – 40%- most commonly gastrinoma (leading to Zollinger-Ellison syndrome), second most common insulinoma
      • Pituitary – 30% 
    • Mutation on chromosome 11 of the gene which codes for menin 
  • MEN 2: 
    • MEN 2a: 
      • Tumours of:
        • Medullary thyroid 
        • Parathyroid 
        • Phaeochromocytoma 
      • Caused by Ret gene on chromosome 10
      • Commonest form of MEN 2 
    • MEN2b
      • Features may include:
        • Medullary thyroid tumour
        • Phaeochromocytoma 
        • Marfanoid habitus 
        • Mucosal neuromas 
        • Megacolon 
      • Caused by Ret gene on chromosome 10

Presentation is with the complications of the tumours, for example hypercalcaemia with parathyroid tumours or hypoglycaemia with insulinoma.
Treatment tends to be surgical.

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