MEN

Question 1

MEN syndrome, to be considered as such has to involve how many endocrine glands?

Answer 1

At least two.

Question 2

True or False, MEN are most often cancerous.

Answer 2

False. they’re mixed of benign and malignant

Question 3

How is MEN inherited and what is it’s penetrance?

Answer 3

Autosomal dominant with high degree of penetrance and variable expressivity

Question 4

Which form of MEN is more common?

Answer 4

MEN 1

Question 5

The fact that MEN can affect so many endocrine organs is explained by _

Answer 5

The tumors arise from amine precursor uptake and decarboxylation (APUD) groups of cells which constitutes the diffuse system of neuroendocrine cells distributed throughout the body.

Question 6

what is meant by APUD and what enzyme is involved?

Answer 6

APUD denotes the capacity of those cells to synthesize and secrete biogeneic amines formed through the activity of the enzyme L-dopa decarboxylase.

Question 7

Hyperparathyroidim is usually NOT seen in which MEN type?

Answer 7

MEN 2B

Question 8

Enteropancreatic tumors are usualy seen in what MEN type?

Answer 8

MEN 1

Question 9

Pituitary adenomas are usuauly seen in what MEN type?

Answer 9

Type 1

Question 10

Medullary thryoid carcinoma are usually seen in what MEN type

Answer 10

Type 2 (both A and B)

Question 11

Pheochormocytomas are usuauly seen in what MEN type?

Answer 11

Type 2 both A and B

Question 12

Mucosal neurons and marfanoid habitus are usually seen in what MEN type?

Answer 12

Type 2B 100%

Question 13

If a patient presents with pancreas/GI endocrine and pituitary adenomas, what MEN type is probably associated?

Answer 13

MEN 1

Question 14

if a patient presents with Pheocromocytomas, medullary thryoid cancer what MEN type is probably associated?

Answer 14

MEN 2

Question 15

If a patient presents with acromegaly, Cushing syndrome, galactorrhea, what is the most likely MEN syndrome assocaited?

Answer 15

MEN 1

Question 16

What is Wermer syndrome?

Answer 16

AKA MEN type 1. It is caused by a mutation in the MEN gene which encodes menin, which normally is a tumor suppressor. So without menin, cell division is unregulated and leads to tumor formation.

Question 17

What MEN is associated with tumors of the three P’s

Answer 17

Type I, which is seen in pituitary, pancreas and parathyroid

Question 18

In MEN type 1, male are like to develop carcoid tumors where?

Answer 18

within the thymus

Question 19

In MEN type 1, females are likely to develop carcinoid tumors where?

Answer 19

Bonchi.

Question 20

How can MEN 1 be diagnosed?

Answer 20

1. Two or more MEN 1 associated tumors
2. Familial: patients with one MEN 1 asosciated tumor and a first-degree relative with MEN 1
3. Genetic: an asymptomatic carrier of MEN 1 mutation

Question 21

In MEN type 1, the most frequently involved organ is _ and tumors will be usually seen in how many of those glands?

Answer 21

Parathyroid glands. On all four glands.

Question 22

GLucognomas, somatostatinomas, VIPomas, and gastrionoma are assoicated with defect in in protein?

Answer 22

those omas describes MEN type 1 which is due to mutation in menin protein.

Question 23

Octreotide is used to treat what condition?

Answer 23

acromegaly

Question 24

Bromocriptine is used to treat what condition?

Answer 24

prolactinomas associated with pituitary tumors

Question 25

MEN type 2 is associated with activating mutation of what gene?

Answer 25

RET protooncogene.

Question 26

What is the normal function of RET?

Answer 26

RET gene encodes for a receptor tyrosine kinase that phosphorylates and activates enzymes critical to cellular development.

Question 27

Medullary thyroid carcinoma is the most common malignant transformation in what type of MEN?

Answer 27

MEN type 2

Question 28

C cell hyperplasia is assoicated with which MEN type disorder?

Answer 28

MEN type 2

Question 29

What is Siplle syndrome?

Answer 29

AKA MEN Type 2A, neoplastic transformation of parathyroids, thyroid parafollicular Cellls and adrenal medulla; pheochromocytomas that secrere greater amount of E then regular pheochormocytomas; and cutaneous lichen amyloidoisis is seen on skiin.

Question 30

How can MEN type 2B be distinguished from MEN type 2A?

Answer 30

THe prsence of MTC and pheochromocytoma with multiple mucosal neuromas in an affected individual. THe MTC is more aggressive and have metastic lesion during infancey. Hyperparathyroidism is NOT seen.

Question 31

List the conditions you’d see in MEN Type 2A

Answer 31

1. medullary thyroid cancer, pheochromocytoma, primary hyperparathryoidism
2. cutnaeous lichen amyloidisis
3. Hirshsprung disease
4. Familial medullay cancer w/o pheochromocytoma or parathyroid hyperplasia

Question 32

List the conditions youd see in MEN Type 2B

Answer 32

1. MTC
2. Pheochromocytoma
3. Mucosal neuromas, intestial gangliooneuromas and marfanoid habitus

Question 33

A patient comes in with 2month hx of hoarseness and occasional palpitation, HA, diaphoresis, and elevated BP. PE shows lumps at base of her neck, biopsy of mass shows C cell hyperplasia, and lab shows hypercalcemia. What is the likely diagnosis?

Answer 33

MEN 2A

Question 34

A baby is born w/o complication to a healthy mom. On PE whos dangerously hypotensive neonate with ambiguous genitalia, fused labia, and an enlarged and mascularized clitoris. what is the likely diagnosis?

Answer 34

CAH, due to 21 beta hydroxylase mutation.