Multiple Endocrine Neoplasia

Question 1

types of MEN from most prevalent to least

Answer 1

MEN1, MEN2A, MEN2B

Question 2

what kind of cells do MEN tumors arise from and what do they do

Answer 2

APUD: can syntheize and or secrete biogenic amines formed through actiity of L-DOPA decarboxylase

Question 3

what is a carcinoid

Answer 3

slow growing type of neuroendocrine tumor

Question 4

what is a carcinoma

Answer 4

cancer related to epithelial cells

Question 5

what is an adenoma

Answer 5

benign, epithelial origin associated with a gland

Question 6

what is a neuroma

Answer 6

tumor in nerve tissue

Question 7

MEN 1 has what tumors (in order of prevelance)

Answer 7

parathyroid adenoma (95%), pancreatic endocrine tumors (50%), pituitary adenoma (40%), carcinoid tumors

Question 8

MEN 2A tumors

Answer 8

medullary thyroid carcinoma, bilateral pheochromocytomas, parathyroid adenoma

Question 9

MEN 2B tumors

Answer 9

medullary thyroid carcinoma, neuromas, phaeochromocytoma

Question 10

mucosal neuromas and marfanoid habitus occur in what disease

Answer 10

MEN 2B

Question 11

MEN I associations

Answer 11

hyperparathyroidism–>hypercalcemia
ZE syndrome
pituitary path: Galactorrhea, Acromegaly, Cushing

Question 12

MEN IIA associations

Answer 12

hyperparathyroidism
hypercalcemia
medullary carcinoma
increased calcitonin
increased catecholamines

Question 13

MEN IIB associations

Answer 13

mucosal nodules
marfanoid body habitus
medullary carcinoma
increased calcitonin
increased catecholamines

Question 14

MEN 1 is caused by mutations in ____ gene which encodes a protein known as ___ which is a ____

Answer 14

MEN1 gene, menin, tumor suppressor

Question 15

MEN1: male patients appear to have predilection for developing ____ tumors
women for developing ____ carcinoids

Answer 15

carcinoid tumors in thymus for men

bronchial carcinoids in women

Question 16

diagnosins MEN 1
clincial
failial
genetic

Answer 16

clinical: 2 or more MEN tumors
familial: 1 MEN tumor and first degree relative with MEN 1
genetic: asymptomatic carrier of MEN1 mutation

Question 17

the most frequent manifestation of MEN 1 pancreatic involvement is what

Answer 17

gastrinoma, then insulinomas

also have glucagonomas, somatostatinomas, VIPomas

Question 18

pituitary tumors of MEN1

-most secrete what and causes what

Answer 18

most secrete prolactin and have hyperprolactinemia (galactorrhea and amenorrhea in women, impotence in men)

Question 19

MEN1 patients may develop tumor secreting __ or __ as well

Answer 19

ACTH and GH

Question 20

treatment for MEN1

Answer 20

surgery

bromocriptine: dopamine agonist, inhibits prolactin
octreotide: somatostatin analogue, inhibits GH secretion by AP
- subtotal pancreatectomy (remove as much tumor as possible)

Question 21

MEN 2 is caused by a mutation in what gene

Answer 21

RET protooncogene that codes for receptor TK

Question 22

what is the most common sign of MEN 2

Answer 22

malignant transformation of parafollicular cells

Question 23

MEN 2A or sipple syndrome parafollicular cell progression

Answer 23

C cell hyperplasia to nodular hyperplasia to malignant degeneration over a variable period

Question 24

hyperparathyroidism is associated with MEN2A and similar to that of MEN-1 but is ____ frequent than MEN1

Answer 24

less

Question 25

what is another skin condition associated with MEN2

Answer 25

cutaneous lichen amyloidosis, itchy skin condition

Question 26

what tumor is not associated with MEN 2B

Answer 26

hyperparathyroidism