Autosomal dominant syndromes.
Two types MEN-I and MEN-II
MEN-I(Wermer Syndrome)
MEN-1 gen
- Pituitary adenoma
- Pancreatic tumours
- Parathyroid hyperplasia
insulinoma,glucogonoma,gastnoma,VIPoma.
Wemer morrison’s syndrome/watery diarrhoea syndrome:Pancreatic diarrhoea
MEN-II
RET gene
MEN-II A(Sipple’s syndrome)
Associated with
- Parathyroid hyperplasia
- Medullary thyroid carcinoma
- pheochromocytoma
MEN -II B
- Medullary thyroid carcinoma(100%)
- Pheochromocytoma
- Marfanoid habitus
- Mucosal neuromas(>95%)
IMPORTANT:Both MEN-I and MEN-IIA have parathyroid hyperplasia as a common point.
MENIIA and MENIIB have Medullary carcinoma and pheochromocytoma as common point.
Wermer described MEN as having autosomal dominant inheritence pattern.
Sipple described a combination of a pheochromocytoma, medullary thyroid carcinoma and parathyroid adenoma.
In 1974 Sizemore et al showed that the MEN 2 category included two groups of patients with MTC and pheochromocytoma: one with parathyroid disease and a normal appearance (MEN 2A) and the other without parathyroid disease but with mucosal neuromas and mesodermal abnormalities (MEN 2B).
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