Multiple Endocrine Neoplasia

Question 1

Describe the two hit theory for MEN syndrome.

Answer 1

Hit 1: gland hyperplasia

Hit 2: tumor formation

Question 2

Name the three glands found in MEN (or MEA) type 1?

Answer 2

1. Anterior Pituitary
2. Parathyroids
3. Pancreas

Question 3

What 3 things are tested in the serum to screen for parathyroid involvement in MEN 1?

Answer 3

1. Calcium
2. Parathyroid hormone
3. Phosphates

Question 4

What type of tumor in a patient with MEN1 is suspected when dermatitis and hypoglycemia are found on PE and testing?

Answer 4

Glucagonoma of the pancreas

Question 5

What type of tumor in a patient with MEN1 is suspected when watery diarrhea and hypokalemia are noted in history and testing?

Answer 5

VIPoma of the pancreas

Question 6

What test would be done to check for anterior pituitary involvement in a patient with MEN 1?

Answer 6

Prolactin levels (searching for hyperprolactinemia)

Question 7

Name the 3 glands often involved in MEN IIA.

Answer 7

1. Thyroid
2. Parathyroid
3. Adrenal

Question 8

What test is done to screen for thyroid involvement in MEN IIA?

Answer 8

Calcitonin levels

Question 9

Name two distinctive features for MEN IIB.

Answer 9

1. Marfanoid Habitus
2. Multiple Ganglioneuromas: easiest to spot when on the skin

(Ganglioneuroma is a tumor of the sympathetic nerve fibers. Normally found in the medulla of adrenal glands, but may also be found elsewhere.)