Multiple endocrine neoplasia

Question 1

What is multiple endocrine neoplasia?

Answer 1

A group of inherited disorders characterised by the development of neoplasms in multiple endocrine organs

Question 2

What is the inheritance of multiple endocrine neoplasia?

Answer 2

Autosomal dominant

Question 3

What genes are affected in multiple endocrine neoplasia?

Answer 3

MEN-1 is caused by mutations in the MEN1 gene

MEN-2a and MEN-2b are caused by mutations in the RET gene

Question 4

What glands are affected in MEN-1?

Answer 4

Parathyroid
Pancreas
Pituitary

Question 5

What glands are affected in MEN-2a?

Answer 5

Thyroid (medullary thyroid cancer)
Adrenal (phaeochromocytoma)
Parathyroid

Question 6

What glands are affected in MEN-2b?

Answer 6

Thyroid (medullary thyroid cancer)
Adrenal (phaeochromocytoma)
Parathyroid
Mucosal neuromas

Question 7

What investigations are involved in the diagnosis of MEN?

Answer 7

Genetic testing
Hormonal assays
Imaging - CT/MRI

Question 8

What is the management of MEN?

Answer 8

Surgical intervention for removal of tumours
Hormonal therapies
Lifelong surveillance for early detection of new tumours