MEN Syndromes

Question 1

MEN syndromes

Answer 1

group of inherited disorders

proliferative lesions
-hyperplasia, adenomas, carcinomas

of multiple endocrine organs

Question 2

MEN1

Answer 2

pituitary
parathyroid
pancreatic

3 Ps

Question 3

MEN2A

Answer 3

parathyroid
medullary thyroid carcinoma
pheochromocytoma

2Ps

Question 4

MEN2B

Answer 4

mucosal neuromas
mafranoid body habitus
medullary thyroid carcinoma
pheochromocytoma

1P

Question 5

think MEN if

Answer 5

young age
multiple endocrine organ tumors
asymptomatic hyperplasia precedes tumor

more aggressive and recur**

Question 6

wermer syndrome

Answer 6

MEN1

pituitary, parathyroid, and pancreas glands

Question 7

MEN1 parathyroid

Answer 7

primary hyperparathyroidism

Question 8

MEN1 pancreas

Answer 8

endocrine tumor of pancreas

aggressive with mets

multiple microadenomas throughout pancreas

often functional tumors - PP most common

Question 9

MEN1 pituitary

Answer 9

most frequently - prolactinoma

Question 10

gastrinomas in MEN1

Answer 10

commonly in duodenum

Question 11

MEN1 mutation

Answer 11

menin

tumor suppressor gene

Question 12

MEN2 mutation

Answer 12

RET protooncogene

GOF mutation on chromosome 10q11.2

Question 13

sipple syndrome

Answer 13

MEN2a

pheochromocytoma, medullary carcinoma of thyroid, parathyroid hyperplasia

Question 14

MEN2b

Answer 14

more aggressive and multifocal medullary thyroid cancers

and pheochromocytomas

no hyperPTH**

also - neuromas or ganglioneuromas (bumps on skin, oral mucosa, eyes, resp tract, GI tract

also - marfanoid habitus

Question 15

RET on genetic testing

Answer 15

prophylactic thyroidectomy

almost all develop medullary carcinoma of thyroid