Overview
Multiple endocrine neoplasia (MEN) describes several syndromes featuring tumours of endocrine organs with different characteristic patterns. MEN is inherited in an autosomal dominant manner.
The main types of MEN are:
- MEN1
- MEN2A
- MEN2B
Features
MEN1
Due to a mutation in the MEN1 gene. Features may be:
- Parathyroid hyperplasia and adenomas:
- Often the presenting feature
- Pituitary adenomas:
- Most commonly prolactinomas
- May lead to acromegaly and Cushing’s syndrome
- Pancreatic endocrine tumours:
- Gastrinomas – recurrent peptic ulcers and Zollinger-Ellison syndrome
- Insulinomas – see Hypoglycaemia
- Angiofibromas
MEN2A
Due to a mutation in the RET oncogene. Features may be:
- Medullary thyroid cancer
- Parathyroid hyperplasia and adenomas
- Phaeochromocytoma
MEN2B
Due to a mutation in the RET oncogene. Features may be:
- Medullary thyroid cancer
- Phaeochromocytoma
- Marfan’s-like body habitus
- Neuromas
- Delayed puberty
Management
- Management involves genetic counselling and surgical removal of affected areas. Replacement hormones are also given.
