Multiple Endocrine Neoplasia (MEN)

Question 1

What is the quintessential hallmark of malignancy?

second hallmark?

Answer 1

metastasis, then invasion

Question 2

Genetics of MEN

• inheritance?
• penetrance/expression?

Answer 2

Autosomal dominant

“MEN are dominant”

-high penetrance and variable expression

Question 3

How can so many various endocrine organs be affected by MEN?

• APUD?
• enzyme?

Answer 3

Question 4

Hyperplasia=?

Hypertrophy=?

what is a neoplasm?

Answer 4

neoplasms persist their secretions even after drugs are given

Question 5

How to tell the three types of MEN apart

-by organ associated tumors

Answer 5

MEN I: Three P’s Parathyroid, Pancreatic (endocrine) tumors, Pituitary

MEN IIa: 2 P’s Parathyroid, Pheochromocytoma

-also has Medullary thyroid carcinoma

MEN IIb: 1P Pheochromocytoma

• also has medullary thyroid carcinoma
• can also be distinguished by musosal neuromas, and marfanoid habitus

Question 6

Gene mutation in Wermer syndrome (MEN I)

Answer 6

menin, which is a tumor suppressor on chromosome 11

Question 7

Carcinoid tumors in MEN I

-male vs female

Answer 7

Question 8

Diagnosis of MEN I

• clinical
• familial
• Genetic

Answer 8

Question 9

-hyperparathyroidism involvement in MEN I and how MEN I is usually detected

Answer 9

95% of MEN I have parathyroidism, but usually symptoms from pituitary or pancreatic involvement are sensed first

Question 10

Hyperparathyroidism with MEN I

-how many glands?

Answer 10

ALL FOUR

Question 11

MEN I endocrine pancreas involvement:

• which manifestation of MEN I?
• first and second most common pancreatic manifestations
• other potential less common pancreatic involvements

Answer 11

• pancreatic involvement overall second most common manifestation of MEN I
• gastrinomas are most frequent (Z-E syndrome)
• insulinomas are second most frequent
• glucagonomas, somatostatinomas, VIPomas

Question 12

• Pituitary involvement in MEN I
• which manifestation?
• prolactin and symptoms in male vs female
• other syndromes associated?

Answer 12

pituitary involvement is third most common

Question 13

Treatment of MEN I

Answer 13

• surgical resection of hyperplastic Parathyroid
• or phamacotherapy of PT via Bromocriptine (prolactinoma) or octreotide (acromegaly)
• remove as many pancreatic tumors as possible

Question 14

MEN 2 mutation

-what does gene code for

Answer 14

-gain of function mutatio of RET protooncogene, which coes for a receptor tyrosine kinase that phosphorylates and activates enzymes critical to cellular development

Question 15

MEN 2

• most common sign
• second most common sign

Answer 15

Question 16

Which type of MEN 2 is more severe?

Answer 16

IIB

Question 17

what is a medullary thyroid carcinoma?

• causes hyperplasia in which type of Cell?
• other peptides and hormones expressed

Answer 17

Causes C cells of thyroid follicles to secrete lots of calcitonin, hyperplasia of C cells

-somatostatin,TRH, VIP, POMC, carcinoembryonic antigen (CEA), and neurotensin

Question 18

Symptoms of pheochromocytoma

Answer 18

uncontrollably high blood pressure non responsive to meds, often times in a patient you wouldnt expect to have such high bp

Question 19

Skin condition associated with MEN IIa

Answer 19

Cutaneous lichen amyloidosis

Question 20

Answer 20

Question 21

MEN IIb

-characteristics

Answer 21

• pheochromocytomas, like in IIa
• but has muscular neuromas, intestinal ganlioneuromas, and a marfanoid body habitus to help distinguish it

Question 22