Multiple endocrine neoplasia (MEN 1, 2a, 2b)

Question 1

What is multiple endocrine neoplasia?

Answer 1

Multiple endocrine neoplasia is a rare syndrome caused by an autosomal dominant gene mutation in a tumour suppressor gene. This causes multiple cancers of the endocrine system to form.There are 3 main types; MEN1, MEN2A, MEN2B

Question 2

What will you find in a history taking of Multiple Endocrine Neoplasia

Answer 2

Symptoms:
MEN 1: parathyroid hyperplasia, insulinoma, pituitary adenomas, gastrinoma (Zollinger Ellison)
MEN 2a: medullary thyroid cancer, parathyroid hyperplasia, pheochromocytoma
MEN 2b: medullary thyroid cancer, pheochromocytoma, also has marfanoid appearance (tall with long limbs) and mucosal neuromas (lips and tongue).

Risk factors:
Family history

Question 3

What investigations will you order in multiple endocrine neoplasia?

Answer 3

Screening for MEN 1:
Serum Calcium - Hyperparathyroidism
Fasting gastrin - Gastrinoma
Prolactin – Pituitary adenoma

Screening for MEN 2:
24 hours urine for catecholamines, metanephrines and vanillyl-mandelic acid - Pheochromocytoma
TFT – Hyperthyroidism
PTH – hyperparathyroid

Question 4

What is the treatment of multiple endocrine neoplasia?

Answer 4

Lifestyle:
Educate the patient about the chronic nature of the disease and potential to pass it onto children.
Screen any first degree relatives

Medical:
Treat each cancer that occurs as you would normally