Master Of Medicine

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).