Multiple Endocrine Neoplasia

Question 1

What is MEN?

Answer 1

A group of inherited disorders

They present in the yuonger age group with multifocal hyperplasia/neoplasms of endocrine organs

Question 2

How does MEN 1 occur?

Answer 2

Mutation in the Men 1 tumour suppressor gene

• > Defective menin protein production
• > Irregular cell growth

Question 3

What are the features of MEN 1?

Answer 3

• Parathyroid Hyperplasia/Adenoma
• Pancreatic tumours (hypoglycaemia)
• Duodenal endocrine tumours (hypoglycaemia & ulcers)
• Pituitary adenomas (mainly prolactinoma)

Question 4

What cause MEN 2?

Answer 4

Mutations in RET proto-oncogenes

Question 5

What are the features of basic MEN 2?

Answer 5

Medullary Carcinoma of thyroid and pheochromocytoma

Question 6

What are the features of the MEN 2A variant?

Answer 6

MEN 2 (medullary carcinoma & pheochromocytoma)

+ Parathyroid hyperplasia

Question 7

What are the features of the MEN 2B variant?

Answer 7

MEN 2 (medullary carcinoma and pheochromocytoma)

+ Neuromas of skin, mucous membrane and skeletal abnormalities.