1) What is it?
- Subgroup of multiple endocrines hyper functions (adenoma or hyperplasia) from mechanisms other than a primary abnormality in the hypothalamic-pituitary axis.
2) Types of MEN
a) MEN 1 (Wermer Syndrome) -4P’
- Parathyroid hyperplasia
- Pituitary adenoma
- Pancreatic adenoma
- With/out PUD (H. Pylori associated Gastritis)
b) MEN 2A (Sipple syndrome)
- Medullary thyroid CA
- Pheochromocytoma
- Parathyroid hyperplasia
c) MEN 2B (mucosal neuroma syndrome)
- Medullary thyroid CA
- Pheochromocytoma
- Mucosal ganglioneuromatosis
- Marfanoid habitus
3) Pathophysiology
- MEN 2A , MEN 2B and familial MTC are autosomal dominant due to germ-line mutation in the RET proto-oncogene located in chromosomes 10 , band q11.2 which codes for a receptor-like tyrosine kinase.
- This mutation convert RET into a dominant transforming gene with oncogenic activity.
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