MULTIPLE ENDOCRINE NEOPLASIA

Question 1

what is multiple endocrine neoplasias (MEN) also known as?

Answer 1

familial endocrine adenomatosis

Question 2

what is multiple endocrine neoplasias (MEN)?

Answer 2

rare group of AUTO DOM inherited syndromes that cause a predisposition to the development of hormone-producing tumors in 2 or more different endocrine glands

tumors may be benign or malignant

Question 3

what are the types of multiple endocrine neoplasias (MEN)?

Answer 3

1. MEN 1
2. MEN 2A
3. MEN 2B
4. MEN 4

Question 4

what is the definition of MEN 1 and what glands does it affect?

Answer 4

development of tumors, or excessive growth and activity, of 2 or more of the following glands:

1. parathyroid glands
2. pancreas
3. pituitary gland
4. thyroid gland
5. adrenal glands
   these 2 are less often affected

Question 5

almost all patients have tumors in what gland in MEN 1?

Answer 5

parathyroid glands

most tumors non-cancerous

Question 6

what is the first clinical manifestation of a patient who has parathyroid gland tumor in MEN 1?

Answer 6

hyperparathyroidism (kidney stones)

Question 7

what are some other common tumors seen in MEN 1?

Answer 7

1. gastrinomas
2. insulinomas
3. pituitary adenomas
4. lipomas and facial angiofibromas (non-endocrine)

Question 8

what is the pathophys of a gastrinoma?

Answer 8

secrete excessive amoutns of gastrin that stimulates overproduction of of stomach acid (peptic ulcers w/ bleeding/perforation)

Question 9

what is the pathophys of a insulinoma?

Answer 9

secrete insulin (hypoglycemia)
tumor with the highest frequency for malignancy

Question 10

what is the definition of a MEN 2A tumor and what does it affect?

Answer 10

development of tumors or excessive growth and activity in 2 or 3 of the following glands:
1. thyroid
2. adrenal (“TAP”)
3. parathyroid

Question 11

what type of cancer do almost everyone with MEN 2A develop?

Answer 11

medullary thyroid cancer

Question 11

what should patients with MEN and diabetes NOT receieve therapy with?

Answer 11

glucagon- like peptide 1 (GLP- 1) agonist because it may increase risk for development of medullary thyroid cancer

Question 12

what happens with overactive parathyroid glands in MEN 2A?

Answer 12

hypercalcemia (kidney stones)

Question 13

what another HUGE common tumors in MEN 2A?

Answer 13

Pheochromocytoma!

Question 14

what is a pheochromocytoma?

Answer 14

a neuroendocrine tumor of the adrenal glands that secretes catecholamine hormones leading to increased adrenaline (high BP, tachycardia, HA, sweating)

often bilat. and noncancerous

Question 15

what is MEN 2B associated with?

Answer 15

marfan-like habitus

(men want 2b tall)

Question 16

what cancer is MEN 2B the develpoment of?

Answer 16

1. medullary thyroid cancer that develops at an early age, grows faster, and spreads more rapidly than those in MEN 2A
2. pheochromocytoma
3. neuromas (mucus membranes)

Question 17

what are neuromas?

Answer 17

glistening bumps around the lips, tongue and lining of mouth
eyelids and lips ay thicken and the lips may turn inside out (evert)

Question 18

which MEN type is the most recently discovered type?

Answer 18

MEN 4

Question 19

what does MEN 4 resemble?

Answer 19

MEN 1, but is caused by a different gene mutation

Question 20

what cancers develop through MEN 4?

Answer 20

1. parathyroid adenoma -> hyperparathyroidism
2. pituitary adenoma
3. pancreatic neuroendocrine tumor
4. gonadal, renal, and/or adrenal tumors

Question 21

what is involved in the diagnosis of MEN?

Answer 21

1. genetic testing- pt. and family members
2. hormone levels (blood and urine)
3. imaging studies to access tumors

Question 22

what is the treatment for MEN?

Answer 22

no cure for any type
tumor removal if possible
total thyroidectomy for all those at risk for medullary thyroid cancer

Question 23

what is the MEN mnemonic?

Answer 23

men 1: 3 P’s
men 2a: 1 M 2 P’s
men 2b: 2M’s 1 P

Question 24

go back to notes

Answer 24

:) fuck you